Download Biochemistry review-ppt

144 Questions (108 slides Q&A)
54:30 timed Qs
Unknown time for answers
158 slides Lecture
~6 hours
Substrate
Catalytic site
Allosteric site
Cofactor
Apoenzyme
Holoenzyme
Metalloenzyme
Zymogen
Isozyme
Enzyme without cofactor
Enzyme with cofactor
Sometimes called coenzyme
One of several enzymes that
catalyze the same reaction
Aka: binding site
Where the reaction takes place
Site where substrates attach
Site where effectors attach
Aka: reactant
Requires an ion for activity
Inactive enzyme
Endergonic or exergonic?
Enzyme
Marker
Full name
Tissue / Organ
ALP
Alkaline phosphatase
Bone or liver
ACP
Acid phosphatase
Prostate
Amylase /
lipase
Pancreas
AST / SGOT
Aspartate aminotransferase Heart and liver
ALT / SGPT
Alanine aminotransferase
Heart and liver
LDH
Lactate dehydrogenase
Heart, liver and
skeletal muscle
CK / CPK
Creatine (phospho) kinase
Heart and skeletal
muscle
IUB Systematic Classification and nomenclature
International Union of Biochemistry (IUB)
Enzymes are classified into 6 classes according to function:
1. Oxidoreductase
Re-dox reactions: A + B=O  A=O + B
2. Transferase
Transfer groups between substrates
3. Hydrolase
Break substrates by addition of water
4. Lyase
Break substrates without water
5. Isomerase
Convert isomers: AB2  B—A—B
6. Ligase
Ligate with new covalent bond: C—C, O, N, S
Synthetase: uses ATP/GTP as energy source
Synthase: uses other energy
IUB Class
Function
Common name
Cofactors
1. Oxidoreductase
Re-dox reactions;
moves hydrogen
Dehydrogenase
Catabolic: NAD and
FAD
Anabolic: NADP
Hydroxylase
Transfers –OH group
Hydroxylase
Vit C and Cu or Fe
2. Transferase
Moves a functional
Transferase
group between two
compounds
Aminotransferase
Swaps amino group
for a ketone group
Aminotransferase
Vit B6 (pyridoxal
phosphate)
Kinase
Transfers PO4
Kinase
ATP and Mg2+
3. Hydrolase
Splits into 2 using
H2O
Substrate + ase
--
4. Lyase
Splits into 2, no H2O
Substrate + ase
--
Removes or adds CO2
(De)carboxylase
Biotin
5. Isomerase
Rearrange molecule
Isomerase
--
6. Ligase
Joins to molecules
Ligase, synthase or
synthetase
--
(De)carboxylase
Enzymes and Coenzymes
Use the following diagram to answer questions 1-6:
(B)
(A)
(C) + 50 kcal
NAD+
(D)
NADH
(E)
ONE MINUTE
To answer all
1. Which one is the product of the reaction
2. Which one is the reduced form of the coenzyme
3. Which one is the oxidized form of the coenzyme
4. Which is the substrate of the reaction
5. Is this reaction reversible or irreversible
6. Is this reaction endergonic or exergonic
45 sec to answer both
7. Pepsin, an enzyme produced
in the stomach to digest
proteins, belongs to which
class of enzyme
a.
oxidoreductase
b.
transferase
c.
hydrolase
d.
lyase
8. To which class of enzyme
does alcohol dehydrogenase
belong:
a. oxidoreductase
b.
transferase
c.
hydrolase
d.
lyase
45 sec to answer both
9. To which class of enzyme
does acetyltransferase
belong:
a. oxidoreductase
b. transferase
c. hydrolase
d. Lyase
10. To which class of enzyme
does glutamine synthetase
belong:
ATP + L-glutamate + NH3 
ADP + Pi + L-glutamine
a.
oxidoreductase
b.
transferase
c.
hydrolase
d.
ligase
1:30 to answer all three
11. The reason enzymes increase the rate of chemical reactions is
because they
a. increase the activation energy required
b. lower the activation energy required
c. increase the free energy of the compounds formed
d. decrease the free energy of the compounds formed
12.
a.
b.
c.
d.
What is true about Vmax
it reflects the concentration of product in a sample
it cannot be affected by the temperature of the reaction
it cannot be affected by the pH of the reaction
it cannot be affected by the substrate concentration
13. Patients who have bone diseases or hepatobiliary diseases
would have a higher concentration of
a.
lactate dehydrogenase
b. alkaline phosphatase
c.
creatine phosphokinase
d. α-amylase
Use the following diagram to answer questions 1-6:
(B)
(A)
(C) + 50 kcal
NAD+
(D)
NADH
(E)
1. Which one is the product of the reaction
C
2. Which one is the reduced form of the coenzyme E
3. Which one is the oxidized form of the coenzyme D
4. Which is the substrate of the reaction
A
5. Is this reaction reversible or irreversible Irreversible
6. Is this reaction endergonic or exergonic
Exergonic
7. Pepsin, an enzyme produced
in the stomach to digest
proteins, belongs to which
class of enzyme
a.
oxidoreductase
b.
transferase
c.
hydrolase
d.
lyase
8. To which class of enzyme
does alcohol dehydrogenase
belong:
a. oxidoreductase
b.
transferase
c.
hydrolase
d.
lyase
9. To which class of enzyme
does acetyltransferase
belong:
a. oxidoreductase
b. transferase
c. hydrolase
d. Lyase
10. To which class of enzyme
does glutamine synthetase
belong:
ATP + L-glutamate + NH3 
ADP + Pi + L-glutamine
a.
oxidoreductase
b.
transferase
c.
hydrolase
d. ligase
11. The reason enzymes increase the rate of chemical reactions is
because they
a. increase the activation energy required
b. lower the activation energy required
c. increase the free energy of the compounds formed
d. decrease the free energy of the compounds formed
12.
a.
b.
c.
d.
What is true about Vmax
it reflects the concentration of product in a sample
it cannot be affected by the temperature of the reaction
it cannot be affected by the pH of the reaction
it cannot be affected by the substrate concentration
13. Patients who have bone diseases or hepatobiliary diseases would
have a higher concentration of
a.
lactate dehydrogenase
b. alkaline phosphatase
c.
creatine phosphokinase
d. α-amylase
Classification of Monosaccharides
- 3 carbon carbs
(trioses)
- 4 carbon carbs
aldoses
ketoses
D-/ L- glyceraldehyde
Dihydroxyacetone
D-/ L- glycerose
D-erythrose
D-erythrulose
D-ribose
D-ribulose
D-xylose
2’-deoxyribose
D-xylulose
-6 carbon carbs
D-glucose
D-fructose
(hexoses)
D-galactose
D-mannose
(tetroses)
- 5 carbon carbs
(pentoses)
Sugar
monomers
bonds
hydrolyzed
Maltose
Dimer of D-glucose (“glucan”) α1-4
maltase
Isomaltose
Dimer of D-glucose
α1-4
isomaltase
Sucrose
D-glucose & D-fructose
α1-2
sucrase (invertase)
Lactose
D-glucose & D-galactose
β1-4
lactase
Cellobiose
Dimer of D-glucose
β1-4
cellulase (bacteria)
Trehalose
Dimer of D-glucose
α1-1
trehalase (microbe)
GLUCOSE
6 carbon carbohydrates
(hexoses)
FRUCTOSE
Aldoses
D-glucose
D-galactose
D-mannose
Ketose
D-fructose
Carb
monomers
Starch
D-glucose
--amylose
--amylopectin branches ~12 res.
bonds
hydrolyzed
α-amylase
α1-4
α1-4 & α1-6
into glucose,
maltose,
isomaltose
Glycogen
D-glucose
branches ~8 res
α1-4 & α1-6
α-amylase
Cellulose
D-glucose: found in plants
β1-4
cellulase
Inulin
D-fructose: found in roots and tubers of lotus, artichokes
and dandilions
Dietary Fibers
Mixture of polysaccharides and non-carbohydrates
Dietary Fibers: intrinsic and intact in natural foods.
Mixture of un-digestible polysaccharides and noncarbohydrate compounds
Cellulose
 Hemicellulose
 Pectin
 Lignin
 Mucilage
 Glycoprotein
 Phytic acid
 Waxes

β14 glucose units
Linear D-xylose w/ side chains
Soluble polymer of galacuronic acid
Methoxy-phenol polymers
Sugar acid polymer and galactan mix
Extensin is the major form
Inositol hexaphosphate, chelating
Esters of fatty acids and alcohols
ATP
ADP
4 ATP made
-2 ATP consumed
2 ATP net gain
Also 2 NADH
hexokinase
phosphofructokinase
ATP
ADP
Glyceraldehyde 3-P
dehydrogenase
Pyruvate kinase
Pyruvate dehydrogenase
GLYCOLYSIS
Glucose  Glucose 6-P
Glucokinase /hexokinase
GLUCONEOGENESIS
Glucose 6-P glucose
Glucose-6 phosphatase
F-6-P  F-1,6-BP
Phosphofructokinase
F-1,6-BP  F-6-P
PEP  Pyruvate
Pyruvate kinase
Pyruvate  oxaloacetate
Pyruvate carboxylase
Fructose-1,6-bisphosphatase
Oxaloacetate PEP
PEP carboxykinase
Glucose
Pyruvate in liver
Post-glycolysis
Pre-Kreb’s
Acetyl-CoA
L-alanine
Lactate
Pyruvate + NAD+  Acetyl CoA + NADH + CO2
Keys to remember:
1. Per glucose, 2 NADH are generated (worth 6 ATP)
2. Pyruvate dehydrogenase “complex” is a regulatory
enzyme
Three enzymes +
five co-factors
Pyruvate dehydrogenase
Vitamins B1, B2
Dihydrolipoyl transacetylase
B3, B5
Dihydrolipoyl dehydrogenase
lipoate
Aldolase B
galactokinase
galactose 1-phosphate
uridyl transferase
Galactosemia:
1. Commonly caused by the deficiency of Gal 1 P uridyl transferase.
2. Sometimes due to a defect of galactokinase
Functions of the PPP:
1. Generate NADPH + H+
for lipogenesis
2. Produce ribose-5-phosphate for nucleotide biosynthesis
3. Alternate ‘shunt’ for glucose metabolism
4. Metabolism of some sugars, e.g. xylitol
The overall reaction for this process is:
Glucose 6-phosphate + 2 NADP+ + H2O →
Ribose 5-phosphate + 2 NADPH + 2 H+ + CO2
Glycogenesis
Key enzymes:
1. Phosphoglucomutase
Isomerizes G—6—P to G—1—P
2. Glycogen synthase
Attaches glucose unit
(from UDP-Glucose) to an existing primer in α14
3. Branching enzyme
a) Removes ~6 glucose chain
from growing glycogen polymer
b) attaches to a nearby glycogen in α16
Glycogenin: needed for synthesis of glycogen primer (‘glycogenesis’)
Key enzymes:
1. Glycogen phosphorylase
Breaks α14 bonds
of glucose in glycogen to form G—1—P
2. Glucan transferase
When a glycogen branch
has been reduced to 4 residues, it removes 3 and
adds to an existing glycogen polymer branch in
α14 linkage
3. Debranching enzyme
Removes final α16 of last glucose residue from
stump to release glucose
Phosphorylates
Dephosphorylates
Glycogen
synthase
Glycogen
phosphorylase
Adrenaline /
Epinephrine
Insulin
+
—
—
+
Phosphorylates
INACTIVE
Phosphorylates
ACTIVE
Dephosphorylates
ACTIVE
Dephosphorylates
INACTIVE
GLUT-1:
Passive-facilitative
Brain, kidney, placenta,
colon, RBCs
GLUT-2:
Passive-facilitative
Liver, pancreatic β cells, kidney
Intestineblood (all mono-)
GLUT-3:
Passive-facilitative
Brain, kidney, placenta
GLUT-4:
ACTIVE
Heart, skeletal ms., adipose
Insulin-stimulated transporter
for active uptake of glucose
GLUT-5:
Passive-facilitative
Intestine
For uptake of pentoses
For hexoses when [high]
Glycosaminoglycans: polymers of carbohydrate
derivatives, especially amino sugars and uronic
acids (ex: hyaluronic acid, chondroitin sulfate and
heparin sulfate)
Peptidoglycan: small peptides with attached GAGs
generally structural components of cell walls
Proteoglycan: proteins with attached GAGs
Glycogen: storage form of glucose in animals
Carbohydrates
1:30 to answer all three
Answer choices for all questions:
a.
NADH
b. NADPH
c.
FADH2
d. Thiamine pyrophosphate
1. Aerobic glycolysis will result in the NET production of?
2. Which is required for the biosynthesis of polyols such as
sorbitol
3. The function of the pentose phosphate pathway results in
the net production of which one
1:30 to answer all three
Answer choices for all questions:
a.
NADH
b. FADH2
c.
GTP
d.
CO2
4. Which is required for the conversion of pyruvate to
oxaloacetate (in gluconeogenesis)
5. Which is required for the action of phosphoenolpyruvate
carboxykinase (in gluconeogenesis)
6. Which is an END product formed by the pyruvate
dehydrogenase complex (pick 2)
1:00 to answer both
7.
a.
b.
c.
d.
Which one catalyzes the conversion of ADP to ATP
D-glyceraldehyde-3-phosphate dehydrogenase
Phosphofructokinase
Lactate dehydrogenase
Pyruvate kinase
8.
a.
b.
c.
d.
Which one catalyzes the conversion of ATP to ADP
D-glyceraldehyde-3-phosphate dehydrogenase
Phosphofructokinase
Lactate dehydrogenase
Pyruvate kinase
1:00 to answer both
9. Which one is used in
glycogenesis
a. Glycogen synthase
b.
Glycogen phosphorylase
c.
Glycogen decarboxylase
d.
Debranching enzyme
10. Which one is responsible for
α1-6 bonding in glycogenesis
a. Glycogen synthase
b.
Glycogen phosphorylase
c.
Branching enzyme
d.
Debranching enzyme
1:30 to answer all three
11. The deficiency of which one is related to galactosemia
a. Glucose-6-phosphate dehydrogenase
b. Pyruvate carboxylase
c. Aldolase B
d. Galactosyl-1-phosphate uridyltransferase
12. FAD is reduced to FADH2 in which one
a. Aerobic glycolysis
b. Anaerobic glycolysis
c. Kreb’s cycle
d. Electron transport chain
13.
a.
b.
c.
d.
Pyruvate is the end product formed in which one
Aerobic glycolysis
Anaerobic glycolysis
Kreb’s cycle
Electron transport chain
1:30 to answer all three
Answer choices for all questions:
a.
Phosphofructokinase
b.
Glyceraldehyde-3-phosphate dehydrogenase
c. Lactate dehydrogenase
d.
Pyruvate kinase
14. Which one catalyzes the formation of NADH in both
aerobic and anaerobic glycolysis
15. In the cell, which one catalyzes the formation of NADH
only under aerobic (not anaerobic) conditions
16. Pi, a low energy metabolite in the body, can stimulate the
activity of which one in an allosteric manner
1:30 to answer all three
Answer choices for all questions:
a.
NADH
b.
NADPH
c. CoASH
d. Thiamine pyrophosphate
17. Which one is required for the conversion of pyruvate to
acetyl-CoA (pick 2)
18. There is a NET production of which one when pyruvate is
converted to acetyl-CoA
19. Which one is required for the conversion of pyruvate to
lactate under anaerobic conditions
1:30 to answer all three
Answer choices for all questions:
a. Aerobic glycolysis
b. Anaerobic glycolysis
c.
Glycogenesis
d.
Glycogenolysis
20. In which pathway is the end product glucose-1phosphate
21. Which pathway is most active under “carbohydrate
loading”
22. Which pathway is most active in the liver when trying to
maintain blood glucose levels
Answer choices for all questions:
a.
NADH
b. NADPH
c.
FADH2
d. Thiamine pyrophosphate
1. Aerobic glycolysis will result in the NET production of?
A
2. Which is required for the biosynthesis of polyols such as
B sorbitol
3. The function of the pentose phosphate pathway results in the
B net production of which one
Answer choices for all questions:
a.
NADH
b. FADH2
c.
GTP
d.
CO2
4. Which is required for the conversion of pyruvate to
D oxaloacetate (in gluconeogenesis)
3 carbon  4 carbon)
5. Which is required for the action of phosphoenolpyruvate
C carboxykinase (in gluconeogenesis)
6. Which is an END product formed by the pyruvate
dehydrogenase complex (pick 2)
A, D
7.
a.
b.
c.
d.
Which one catalyzes the conversion of ADP to ATP
D-glyceraldehyde-3-phosphate dehydrogenase
Phosphofructokinase
Lactate dehydrogenase
Pyruvate kinase
8.
a.
b.
c.
d.
Which one catalyzes the conversion of ATP to ADP
D-glyceraldehyde-3-phosphate dehydrogenase
Phosphofructokinase
Lactate dehydrogenase
Pyruvate kinase
9. Which one is used in glycogenesis
a. Glycogen synthase
b. Glycogen phosphorylase
c. Glycogen decarboxylase
d. Debranching enzyme
10. Which one is responsible for α1-6 bonding
in glycogenesis
a. Glycogen synthase
b. Glycogen phosphorylase
c. Branching enzyme
d. Debranching enzyme
11. The deficiency of which one is related to galactosemia
a. Glucose-6-phosphate dehydrogenase
b. Pyruvate carboxylase
c. Aldolase B
d. Galactosyl-1-phosphate uridyltransferase
12. FAD is reduced to FADH2 in which one
a. Aerobic glycolysis
b. Anaerobic glycolysis
c. Kreb’s cycle
d. Electron transport chain
13.
a.
b.
c.
d.
Pyruvate is the end product formed in which one
Aerobic glycolysis
Anaerobic glycolysis
Kreb’s cycle
Electron transport chain
Answer choices for all questions:
a.
Phosphofructokinase
b.
Glyceraldehyde-3-phosphate dehydrogenase
c. Lactate dehydrogenase
d.
Pyruvate kinase
14. Which one catalyzes the formation of NADH in both
B aerobic and anaerobic glycolysis
15. In the cell, which one catalyzes the formation of NADH
C only under aerobic (not anaerobic) conditions
16. Pi, a low energy metabolite in the body, can stimulate the
A activity of which one in an allosteric manner
Pi = low energy state
Regulates one of the key enzymes of glycolysis
Answer choices for all questions:
a.
NADH + H+
b.
NADPH + H+
c. CoASH
d. Thiamine pyrophosphate
17. Which one is required for the conversion of pyruvate to
acetyl-CoA (pick 2)
C, D
18. There is a NET production of which one when pyruvate is
converted to acetyl-CoA
A
19. Which one is required for the conversion of pyruvate to
lactate under anaerobic conditions
A
Answer choices for all questions:
a. Aerobic glycolysis
b. Anaerobic glycolysis
c.
Glycogenesis
d.
Glycogenolysis
20. In which pathway is the end product glucose-1phosphate
D
21. Which pathway is most active under “carbohydrate
loading”
C
22. Which pathway is most active in the liver when trying to
maintain blood glucose levels
D
Isocitrate
Keys
3 NADHs get made
1 FADH2 gets made
1 GTP gets made
Acetyl-CoA gets
consumed
Both CO2 come from
acetyl-CoA
ENZYME
ACTIVATOR
INHIBITOR
Citrate Synthase
High NAD
High ATP
Isocitrate
Dehydrogenase
High ADP
High ATP and NADH
α-ketoglutarate
dehydrogenase
CoASH, pyruvate,
high ADP
Fatty acids, ketone bodies,
High NADH
Succinate thiokinase Mg2+
/dehydrogenase
Oxaloacetate
1. Occurs in the mitochondria
2. Functions under aerobic conditions only
3. Two carbons are oxidized to CO2, and the energy
from these reactions is stored in GTP, NADH and
FADH2
4. NADH and FADH2 are coenzymes that store
energy and are utilized in oxidative
phosphorylation (ETC)
5. Because TWO acetyl-CoA molecules are produced
from each glucose molecule, two cycles are
required per glucose.
6. At the end of all cycles from one glucose, the products
are two GTP, six NADH, two FADH2, four CO2.
7. The cycle is regulated by allosteric inhibition at rest
8. NADH is produced by dehydrogenase reactions
9. FADH2 is produced by succinate dehydrogenase
10. GTP is formed by succinyl-CoA synthetase
Complex I
Complex III
Complex IV
Complex II
Keys of Electron Transport Chain (ETC):
1.
ETC members are embedded in the inner mitochondrial
membrane
2.
NADH is the primary electron donor
3.
Oxidation of each NADH yields 3 ATP, each FADH2
yields 2 ATP
4.
Complexes I, III and IV are proton pumps, while Q and
cytochrome c are mobile electron carriers.
5.
ATP is generated from ADP + Pi by ATP Synthase
6.
No direct regulation of the ETC
Enzyme
Reaction
catalyzed
Pathway
Molecule
generated
Phosphoglycerate
kinase
1,3-BPG to 3PG
Glycolysis
ATP
Pyruvate kinase
PEP to pyruvate
Glycolysis
ATP
Succinyl-CoA
synthetase
Succinyl-CoA to
succinate
Kreb’s cycle
GTP
ATP synthase
ADP to ATP
ETC
ATP
Creatine
(phospho)kinase
Phosphocreatine to Skeletal
creatine
muscle and
brain
ATP
Energetics
1:30 to answer all three
1. The coenzyme form of which ones are involved in oxidative
phosphorylation (pick 2)
a. Vitamin B1
b. Vitamin B2
c. Vitamin B3
d. Vitamin B6
2. The Kreb’s cycle results in a NET production of which
ones (pick 2)
a. NADH
b. NADPH
c. FADH2
d. Thiamine pyrophosphate
3. Which is the first enzyme in the Kreb’s cycle. It catalyzes the
reaction between Acetyl-CoA and oxaloacetate
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
1:30 to answer all four
Answer choices for all questions
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
4. Which one catalyzes the formation of GTP
5. Which one catalyzes the formation of FADH2
6. Which one catalyzes the formation of NADH
7. Which catalyzes the formation of CO2
1:30 to answer all three
8. Mg2+ is required for the
activity of which one
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
9. Which can be formed by the
action of malate
dehydrogenase
a. Acetyl-CoA
b. GTP
c. NADH
d. FADH2
10. The carbons in the CO2
released in the Kreb’s cycle
were originally in which
molecule
a. Acetyl-CoA
b. GTP
c. NADH + H+
d. FADH2
1. The coenzyme form of which ones are involved in oxidative
phosphorylation (pick 2)
a. Vitamin B1
b. Vitamin B2
c. Vitamin B3
d. Vitamin B6
2. The Kreb’s cycle results in a NET production of which
ones (pick 2)
a. NADH
b. NADPH
c. FADH2
d. Thiamine pyrophosphate
3. Which is the first enzyme in the Kreb’s cycle. It catalyzes the
reaction between Acetyl-CoA and oxaloacetate
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
4. Which one catalyzes the
formation of GTP
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate
dehydrogenase
d. succinate thiokinase
5. Which one catalyzes the
formation of FADH2
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate
dehydrogenase
d. succinate thiokinase
6. Which one catalyzes the
formation of NADH
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
7. Which catalyzes the formation
of CO2
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
8. Mg2+ is required for the activity
of which one
a. citrate synthase
b. succinate dehydrogenase
c. α-ketoglutarate dehydrogenase
d. succinate thiokinase
9. Which can be formed by the
action of malate dehydrogenase
a. Acetyl-CoA
b. GTP
c.
NADH
d.
FADH2
10. The carbons in the CO2
released in the Kreb’s cycle
were originally in which
molecule
a. Acetyl-CoA
b.
GTP
c.
NADH + H+
d.
FADH2
1. Triglycerides
Fat and oil
2. Phospholipids
Lecithin, etc
3. Isoprene lipids
Carotenoids, Vit A, etc
4. Steroid lipids
Cholesterol, etc
5. Sphingolipids
Sphingomyelin, glycolipids
6. Lipid derivatives
Eicosanoids, lipoprotein,
etc

Saturated (SAFA):


Possess ONLY single bonds between carbons
Mono unsaturated (MUFA):


Primarily in animal fats
50:50
Possess ONE double bond per molecule
Polyunsaturated (PUFA):Primarily in vegetable oils

Possess at least two double bonds between carbons
Exceptions: plant oils with little PUFA
Coconut oil
High in medium-chain fatty acids
C10-C14
Olive oil
High in oleic acid, a C18 MUFA
C18:1,9
PUFAs that cannot be synthesized by the human
body
linoleic acid and α-linolenic acid
“LA” and “ALA”
PUFAs not synthesized in significant amounts
γ-linolenic acid, arachidonic acid
and eicosapentaenoic acid
“GLA” “AA” “EPA”
C20:3;8,11,14 (DGLA)
ω-6
PG1
LT3
1
3
C20:4;5,8,11,14 (AA)
PG2
LT4
PGI2
TX2
2
4
2
2
PG3
LT5
PGI3
TX3
3
5
3
3
ω-6
C20:5,5,8,11,14,17 (EPA)
ω-3
Lipoproteins are responsible for transport of lipids
Lipoprotein
Chylomicron
VLDL
IDL
Carry TGs from intestines to liver
and adipose
Carry newly synthesized TGs from
liver to adipose
↑ FC and CE
LDL
↑ FC and CE; carry cholesterol from
liver to other tissues; “Bad cholesterol”
HDL
Scavenges cholesterol from body’s
tissues/blood to liver; “Good
cholesterol”
FFA, monoacylglycerol, glycerol, cholesterol and
lysophospholipids
 enter intestinal mucosal cells
 re-esterify to form TG, PL and CE
 aggregate with apolipoproteins and
free cholesterol to form chylomicrons
 released by exocytosis into the lymph
 Blood
 transported to liver, adipose, cardiac & skeletal ms
 catabolized by lipoprotein lipase into VLDLs
Lipoprotein lipase
at peripheral tissue…
activated by PL and apo-C
remnant
To get LDL into peripheral tissues, the tissues must
have a receptor: LDL receptor
apo-C, apo-E
Nascent VLDL
apo-C
VLDL
apo-C, apo-E
IDL
LDL
Tissues
E: Endosome
L: Lysosome
ACAT: Acyl-CoA-cholesterol
acyl transferase
Primarily produced by liver (some in sm. intestine)
Circulating HDL picks up free cholesterol from peripheral
tissues
PUFA from lecithin
Free cholesterol
Cholesteryl ester
LCAT:
Lecithin cholesterol acyl transferase
Transfer cholesteryl
esters to LDL or
chylomicron
remnants for
deposition in
tissues
increase in size
as they circulate
through the
bloodstream by
incorporating
more cholesterol
sequestered into
the core of the
HDL lipoprotein
Adipose Triglycerides are mobilized by
Hormone-Sensitive Triglyceride Lipase
(“Hormone-sensitive lipase”)
TG
HSL
lipolysis
Free Fatty acid + Diacylglycerol
Diacylglycerol
Other enzymes
lipolysis
Free fatty acids are released from the cell
 bind serum albumin
 transported in blood
 liver (and other tissues)
 utilized for energy
Free fatty acids + glycerol
lipoprotein lipase:
cleaves lipoproteins (VLDL, chylomicrons)
releases triglycerides for tissues
phospholipase A2:
cleaves phospholipids (and sphinogmyelins)
releases fatty acids for eicosanoid synthesis
hormone-sensitive lipase:
cleaves stored fats (adipocytes)
releases fatty acids for gluconeogenesis
acetyl-CoA carboxylase:
converts acetyl-CoA to malonyl-CoA for fatty acid
synthesis
Acyl-CoA + CoASH + FAD + NAD + H2O
Acetyl-CoA + Acyl-CoA + FADH2 + NADH
The FADH2 + NADH are further oxidized in the
electron transport chain for ATP formation
Note:
If EVEN # of carbons in fatty acid
If ODD # of carbons in fatty acid
Acetyl-CoA
Propionyl-CoA

Transport of Acyl-CoA into mitochondria using carnitine
as a carrier
CPT I
CPT II
Glucose
Malonyl-CoA
3C
CO2
ADP
ATP
CO2
acyl-CoA
Cn+2
Acetyl-CoA
2C
ATP- Citrate
lyase
Citrate
Protein
CoASH
3-ketoacyl-CoA
Cn+2
2 NADPH + H+
acyl-CoA
Cn+2
2 NADP + H2O
 Desaturase
Systems: Enzymes responsible for creating
double bonds

The desaturase functions with the elongase system to
synthesize longer unsaturated fatty acids
 Regarding
PUFAs: Humans can only place double bonds
between the carboxyl group and the first double bond
 Not between the first double bond and the last carbon
 Ketone
Bodies
Acetone
 β-hydroxybutyrate
 Acetoacetate

 Although
ketogenic amino acids can be converted to
ketone bodies, most come from β-oxidation
Excessive β-oxidation  acetoacetyl-CoA + acetyl-CoA
HMG-CoA
HMG-CoA Synthase
HMG-CoA Lyase
Acetone
β-hydroxybutyrate
Acetoacetate + Acetyl-CoA
CYTOPLASM
MITOCHONDRIA
2 Acetyl-CoA
CoASH
+ Acetoacetyl-CoA
Acetyl-CoA
CoASH
CoASH +
Mevalonate
ATP + Mg2+ &
decarboxylation
HMG-CoA
reductase
2 NADP
HMG-CoA
2 NADPH + H+
6 Isopentenyl pyrophosphate
(5C)
Cholesterol
(27C)
Key Regulatory Enzyme: HMG-CoA reductase
Decreased activity
1. Fasting
2. Cholesterol
3. Dietary cholesterol
4. Statins (analog drugs of mevalonate) … like
Lipator
5. Glucagon and glucocorticoids
Increased activity
1. Insulin

Multiple sclerosis:
 Lack of sphingolipids in white matter
 Cholesteryl esters found in white matter
 CSF has elevated levels of phospholipids
 Deficiency of GABA

Niemann-Pick disease:
 Deficiency of sphingomyelinase


Signs: enlarged liver and spleen, MR, fatal early in life
Tay-Sach’s disease:
 Defect of hexoseaminidase  build up of gangliosides

Signs: MR, blindness, muscular weakness
Lipids
1:30 to answer all four
Answer choices for all questions:
a. Acetyl-CoA
b. CO2
c. NADPH
d. ATP
1. Which one is produced in the tricarboxylic acid cycle?
2. Which one is produced in the pentose phosphate pathway?
3. A large amount of which one is produced in the electron
transport chain?
4. Which one is the direct chief energy source for biochemical
reactions?
1:30 to answer all three
Answer choices for all questions:
a. Linoleic acid
b. Arachidonic acid
c. Butyric acid
d. Eicosapentaenoic acid
5. Which one DOES NOT lead to the production of
malondialdehyde?
6. The eicosanoids derived from which one is good for
preventing heart attack and stroke?
7. The eicosanoids derived from which one are mediators
of allergic reactions and thrombosis?
1:30 to answer all four
Answer choices for all questions:
a. Chylomicrons
b. VLDL
c. LDL
d. HDL
8. Which one is primarily responsible for removing peripheral
cholesterol back to the liver for metabolism
9. Which one is responsible for the transport of dietary lipids
from the intestine into the body tissues
10. Which one is primarily responsible for the transport of
endogenously synthesized lipids from the liver to the
peripheral tissues
11. The receptor for which one is defective in the liver of
familial hypercholesterolemia victims
2:00 to answer all four
Answer choices for all questions:
a. Pancreatic lipase
b. Lipoprotein lipase
c. Hormone-sensitive lipase
d. Phospholipase A2
12. Anti-inflammatory steroids work by inhibiting which one?
13. Which one is responsible for the digestion of dietary lipids
in the gut?
14. Which one is essential for the transfer of lipids from blood
into cells?
15. Which one is responsible for the lipolysis of triglycerides
in adipocytes?
2:30 to answer all four
16. When palmitoyl-CoA is
oxidized through β-oxidation,
eight molecules of which one
are formed? (pick 2)
a. Thiokinase
b. Carnitine
c. NADH + H+
d. Acetyl-CoA
18. In the fed state, high dietary fat
does not cause ketosis because
activity of which one is
inhibited by malonyl-CoA?
a. Acetyl-CoA carboxylase
b. Carnitine-palmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
17. ATP and Mg2+ are required for
the activity of which one?
a. Acetyl-CoA carboxylase
b. Carnitine-palmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
19. Acetoacetate is synthesized in
which pathway?
a. Glycolysis
b. β-oxidation
c. Lipogenesis
d. Ketogenesis
Answer choices for all questions:
a. Acetyl-CoA
b. CO2
c. NADPH
d. ATP
B
C
1. Which one is produced in the tricarboxylic acid cycle?
2. Which one is produced in the pentose phosphate pathway?
3. A large amount of which one is produced in the electron
D transport chain?
4. Which one is the direct chief energy source for biochemical
D reactions?
Answer choices for all questions:
a. Linoleic acid
b. Arachidonic acid
c. Butyric acid
d. Eicosapentaenoic acid
5. Which one DOES NOT lead to the production of
malondialdehyde?
C
6. The eicosanoids derived from which one is good for
preventing heart attack and stroke?
D
7. The eicosanoids derived from which one are mediators
of allergic reactions and thrombosis?
B
Answer choices for all questions:
a. Chylomicrons
b. VLDL
c. LDL
d. HDL
8. Which one is primarily responsible for removing peripheral
cholesterol back to the liver for metabolism
D
9. Which one is responsible for the transport of dietary lipids
A from the intestine into the body tissues
10. Which one is primarily responsible for the transport of
B endogenously synthesized lipids from the liver to the
peripheral tissues
11. The receptor for which one is defective in the liver of
C familial hypercholesterolemia victims
Answer choices for all questions:
a. Pancreatic lipase
b. Lipoprotein lipase
c. Hormone-sensitive lipase
d. Phospholipase A2
12. Anti-inflammatory steroids work by inhibiting which one?
D
13. Which one is responsible for the digestion of dietary lipids
A in the gut?
14. Which one is essential for the transfer of lipids from blood
B into cells?
15. Which one is responsible for the lipolysis of triglycerides
C in adipocytes?
16. When palmitoyl-CoA is
oxidized through β-oxidation,
eight molecules of which one are
formed?
a. Thiokinase
b. Carnitine
c. NADH + H+
d. Acetyl-CoA
17. ATP and Mg2+ are required for
the activity of which one?
a. Acetyl-CoA carboxylase
b. Carnitine-palmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
18. In the fed state, high dietary
fat does not cause ketosis
because activity of which one
is inhibited by malonyl-CoA?
a. Acetyl-CoA carboxylase
b. Carnitinepalmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
19. Acetoacetate is synthesized
in which pathway?
a. Glycolysis
b. β-oxidation
c. Lipogenesis
d. Ketogenesis

“Essential” Amino Acids cannot be
synthesized by the body.










Phenylalanine
Valine
Threonine
Tryptophan
Isoleucine
Methionine
Histidine
Arginine
Leucine
Lysine
(Phe, F)
(Val, V)
(Thr, T)
(Trp, W)
(Ile, I)
(Met, M)
(His, H)
(Arg, R)
(Leu, L)
(Lys, K)
PVT TIM HALL
Adults can synthesize
Adults can synthesize
 Aliphatic:
Gly, Ala, Val, Leu, Ile
 Hydroxyl:
Ser, Thr, Tyr, Hyl, Hyp
 Sulfer:
Cys, Met
 Carboxyl/amide: Asp, Glu / Asn, Gln
 Basic:
Lys, Arg, His, Hyl
 Aromatic:
His, Phe, Tyr, Trp
 Pyrrolidine: Pro, Hyp
Pyrrolidine
 Amide
bond: (aka peptide), between COOH and
NH3. Catalyzed by enzymes
 Glycosidic
bond: proteins covalently attached
to carbohydrates
 Zwitterion:
molecule with both positive and
negative charges
 Isoelectric
point: when the sum of all positive
charges equals the sum of all negative charges.
Net zero charge
Catechol
Catecholamine No longer an
amino acid
Fate of pyruvate
Glucose
Pyruvate in liver
Post-glycolysis
Pre-Kreb’s
Acetyl-CoA
L-alanine
Lactate
1. Glutathione (GSH): intracellular antioxidant
2. Thyrotropin-releasing hormone (TRH):
tripeptide from hypothalamus, stimulates pituitary
3. Substance P: Pain neurotransmitter
4. Kinins (Bradykinin and Kallidin): vasodilating
5. Opiopeptides: analgesic action
Enkaphalin
Beta-endorphin
Dynorphin
Orexigenic: Peptides that INCREASE appetite
Released by






Agouti-related peptide
Neuropeptide Y
Melanin Concentrating Hormone (MCH)
Orexins (hypocretins)
Insulin
Ghrelin
Brain
Brain
Brain
Brain
Pancreas
Stomach
Anorexigenic: Peptides that DECREASE appetite
Released by





Peptide YY: blocks ghrelin
Glucagon-like peptide: delays stomach
emptying, acts on satiety center in brain
Cholecystokinin
Melanocortin: binding R inhibits intake
Leptin: regulates intake of kcal, C, F,
but not percentages of each
Does not prevent obesity
Intestine
Intestine
Intestine
Brain
Adipocyte
 Primary



Structure: Specific amino acid sequence
Connected by peptide bond (covalent amide bond) which can
be hydrolyzed by proteases or boiling
Primary structure is dictated by codons in genes
Primary structure dictates subsequent organization
 Secondary




Structure:
α-helix
β-pleated sheet
β-bending
Random coil
H-bonding
 Tertiary



Structure: 3-D structure of protein
3-D structure related to function
Examples: the enzyme catalytic site which forms a pocket for
a specific substrate
H-bonds, ionic bonds, hydrophobic interactions, and disulfide
bonds
 Quaternary



Structure:
More than 1 polypeptide subunit (aka monomer)
H-bonds, ionic bonds, hydrophobic interactions
Degree of subunit association affects function
Collagen



Most abundant protein in the body
Mature tropocollagen composed of 3 α-chain subunits held
together by H-bonds
Glycine is the most abundant amino acid in collagen, with lots of
proline and lysine
Hydroxylation of
Proline by prolyl hydroxylase and
Lysine by lysyl hydroxylase
Req’s Fe, Vit C, and O2
Histidine  histamine
Phenylalanine  tyrosine  catecholamines
1. dopamine
2. norepinephrine
3. epinephrine
Tryptophan  serotonin
Tyrosine  melanins
Amination (NH4+) or Amidation (NH2)
synthesis of aspartatic acid and glutamic acid
NADPH + H+
Oxaloacetate+
NADP
Aspartate
NH4+
NADH + H+
KREB’s
CYCLE
H2O
NAD
Glutamate
dehydrogenase
NADPH + H+
α-ketoglutarate +
NADP
Glutamate
NH4+
NADH + H+
H2O
NAD
Classified into the following reaction types:
Amination (NH4) or Amidation (NH2)
synthesis of asparagine and glutamine
Glutamate+
NH4+ +
ATP
Glutamine
synthetase
Glutamine +
ADP
+ Pi
Mg2+
Aspartate+
NH4+
+
ATP
Asparagine
synthetase
Asparagine +
ADP
+ Pi
Classified into the following reaction types:
Transamination with transaminases and B6PO4
Pyruvate
+
Alanine
NH2
Glutamate
Oxaloacetate + NH2
Glutamate
3-P-glycerate + NH2
Glutamate
aminotransferase
Alanine + α-ketoglutarate
B6PO4
Aspartate
aminotransferase
3 Steps
Aspartate + α-ketoglutarate
Serine
The left over amino acids
a. Serine  Glycine
b. Glutamate  Proline
c. Methionine + Serine  Cysteine
d. Aspartate + Glutamine  Asparagine
e. Urea cycle  Arginine
1. The urea cycle consists of five reactions
- two mitochondrial and three cytosolic.
2. Requirements for Urea synthesis:
The cycle converts two amino groups
(one from NH4+ and one from Asp)
3. Cost: four "high-energy" phosphate bonds
(3 ATP hydrolyzed to 2 ADP and one AMP).
Summary reaction:
NH3 + aspartate + HCO3- + 3 ATP → urea + fumarate + 2 ADP + 1AMP + 4 Pi
(2nd NH3)(4 bonds)
4. The Urea Cycle is the site of Arginine synthesis
Branch-chain amino acids
Amino group
Pyruvate
Alanine
Alanine
LIVER
deamination
NH3
UREA
glycolysis
Glucose
Glucose
gluconeogenesis
Pyruvate
Maple syrup urine disease:
Defect in metabolism of branch-chain amino acids
Phenylketonuria:
Genetic deficiency of phenylalanine hydroxylase
Alkaptonuria (Dark urine disease):
Tyrosine metabolism is interrupted
Primary hyperoxaluria:
Vitamin B6 deficiency inhibits glycine transaminase
Sulfite oxidase dysfunction:
Cysteine amino acid metabolism is disrupted
Albinism:
Defect in tyrosinase
Glycine + succinyl CoA    Protoporphyrin III
Protoporphyrin III + Fe2+ = Heme
Hyperbilirubinemia:
When blood [bilirubin] > 1 mg/dL
When blood [bilirubin] > 20-25 mg/dL, it penetrates tissues
and results in jaundice (icterus)
Unconjugated hyperbilirubinemia (Retention type):
Not conjugated to glucuronate. No bilirubin detected in urine
Conjugated hyperbilirubinemia (Regurgitation type):
Conjugated to glucuronate to form bilirubin diglucuronate
Small peptides are further digested to amino
acids in the intestine wall by peptidases
 Disaccharides are digested in the small intestine
by dissacharidases (e.g. maltase)


Dietary Guidelines
0.8g/kg body weight
 1.2g/kg during pregnancy and lactation
 12-15% total daily kcal

Soybean
Nuts
Peanut
Other
Legume
-
+
-
+
-
+
-
Blank:
adequate
+
+
-
+ High
-Low
+
Gelatin
Green
leafy
veggies
Sesame
Sunflower
seed
Grain
Cereal
-
Meat
Eggs
Dairy
-
Met
Ile
Leu
Lys
Phe
Thr
Trp
Val
+
+
+
+
+
+
+
-
-
+
EAA
 Consequence:

Kwashiorkor: “Disease of the first child”



Protein-Energy Malnutrition (PEM)
Due to chronic protein deficiency
Signs/Sx: Weakness, edema, loss of hair, skin lesions,
diarrhea, fatty liver, recurrent infections (MCC death is
infection).
Marasmus: Total deficiency of all ergogenic
macronutrients (C, P, F)

No edema. Leads to total loss of body mass
 Diabetes
(insulin resistance)
 Hypercalciuria
 Osteoporosis
 Elevated
(kidney stones)
(bone resorption)
BUN (Blood Urea Nitrogen)
Amino Acids and Protein
1:30 to answer all four
Answer choices for all questions:
a.
Lysine
b. Tyrosine
c. Tryptophan
d.
Glycine
1. Which one is a nutritionally essential amino acid?
2. Which one is ketogenic only (it cannot be glycogenic)?
3. L-DOPA, a drug for Parkinson’s disease, is actually
synthesized in the body from which one?
4. Serotonin, a neurotransmitter related to sleep, is synthesized
in the body from which precursor?
1:30 to answer all four
Answer choices for all questions:
a.
Lysine
b. Tyrosine
c. Tryptophan
d.
Glycine
5. Catecholamines are synthesized from which precursor
6. The nitrogen atom of heme is derived from which amino
acid
7. The skin pigment melanin is derived from which precursor
amino acid
8. Adrenaline is derived from which one
2:00 to answer all four
Answer choices for all questions:
a.
Glutamate dehydrogenase
b. Alanine aminotransferase
c.
Prolyl hydroxylase
d.
Glutamine synthase
9. Which one cannot function without a coenzyme derived from
vitamin B6
10. Which one cannot function without vitamin C
11. Which one cannot function without a coenzyme derived
from niacin
12. The action of which one is critically related to the formation
of mature collagen
1:30 to answer all three
Answer choices for all questions:
a.
Hyperammonemia
b. Alkaptonuria
c.
Phenylketonuria
d.
Maple syrup urine disease
13. Which one could be caused by a deficiency in urea cycle
enzymes?
14. Which one is due to the defect of the enzyme responsible for
the metabolism of the α-keto acids of the branch chain
amino acids?
15. Which one is due to the defect of homogentisate oxidase, an
enzyme involved in the catabolism of tyrosine?
1:30 to answer all three
Answer choices for all questions:
a.
Hyperammonemia
b. Alkaptonuria
c.
Phenylketonuria
d.
Maple syrup urine disease
16. Which one is due to the deficiency of phenylalanine
hydroxylase
17. Which one is due to the deficiency of glycine transaminase
18. Which one can lead to black bone and dark urine
1:30 to answer all three
Answer choices for all questions:
a.
Prolyl hydroxylase
b. Carbamoyl phosphate synthase
c. Arginase
d.
L-α-amino acid oxidase
19. Vitamin C and Fe2+ are cofactors for which one
20. Which one catalyzes the deamination of amino acids to
form ammonia
21. Deficiency of which one is most likely to result in
hyperammonemia
Answer choices for all questions:
a.
Lysine
b. Tyrosine
c. Tryptophan
d.
Glycine
1. Which one is a nutritionally essential amino acid?
A
2. Which one is ketogenic only (it cannot be glycogenic)?
A
3. L-DOPA, a drug for Parkinson’s disease, is actually
B synthesized in the body from which one?
4. Serotonin, a neurotransmitter related to sleep, is synthesized
C in the body from which precursor?
Answer choices for all questions:
a.
Lysine
b. Tyrosine
c. Tryptophan
d.
Glycine
5. Catecholamines are synthesized from which precursor
B
6. The nitrogen atom of heme is derived from which amino
D acid
7. The skin pigment melanin is derived from which precursor
B amino acid
8. Adrenaline is derived from which one
B
Answer choices for all questions:
a.
Glutamate dehydrogenase
b. Alanine aminotransferase
c.
Prolyl hydroxylase
d.
Glutamine synthase
9. Which one cannot function without a coenzyme derived from
vitamin B6
B
10. Which one cannot function without vitamin C
C
11. Which one cannot function without a coenzyme derived
from niacin
A
12. The action of which one is critically related to the formation
of mature collagen
C
Answer choices for all questions:
a.
Hyperammonemia
b. Alkaptonuria
c.
Phenylketonuria
d.
Maple syrup urine disease
13. Which one could be caused by a deficiency in urea cycle
enzymes?
A
14. Which one is due to the defect of the enzyme responsible for
the metabolism of the α-keto acids of the branch chain
D
amino acids?
15. Which one is due to the defect of homogentisate oxidase, an
B
enzyme involved in the catabolism of tyrosine?
Answer choices for all questions:
a.
Hyperammonemia
b. Alkaptonuria
c.
Phenylketonuria
d.
Maple syrup urine disease
16. Which one is due to the deficiency of phenylalanine
hydroxylase
C
17. Which one is due to the deficiency of glycine transaminase
A
18. Which one can lead to black bone and dark urine
B
Answer choices for all questions:
a.
Prolyl hydroxylase
b. Carbamoyl phosphate synthase
c. Arginase
d.
L-α-amino acid oxidase
19. Vitamin C and Fe2+ are cofactors for which one
A
20. Which one catalyzes the deamination of amino acids to
form ammonia
D
21. Deficiency of which one is most likely to result in
hyperammonemia
B
5’-phosphate
2’-deoxyribose
phosphodiester bond:
connects monomers together
H
3’-hydroxyl
Purines
 Possess
a 2-ring heteronitrogen structure
 Adenine (A)
Occur in nucleic
 Guanine (G)
acids
 Xanthine (X)
or their
metabolites
 Hypoxanthine (Hx)
 Caffeine
 Theophylline
 Theobromine
Occur in food
Pyrimidines
 Possess
a 1-ring heteronitrogen structure
 Cytosine (C)
Found in both RNA and DNA
Found in RNA only
 Uracil (U)
 Thymine (T)
Found in DNA only
 RNA
has ribose
 DNA has deoxyribose
 Nucleoside: nitrogen base + ribose
 Nucleotide: nucleoside + phosphate (1, 2, or 3)
Synthesis of all nucleotides
BASE + Phosphoribose
as “PRPP”
PRPP: Phosphoribosyl Pyrophosphate
 DNA




Antiparallel double helix
Hydrogen bonds
A binds with T (2 H-bonds)
C binds with G (3 H-bonds)
 Replication


is “semi-conservative”
Begins with RNA primer
DNA is always synthesized 5’  3’
 Contains
the codons for directing the sequence of the
amino acids of a polypeptide (protein)
 Each
mRNA has a cap (head) which is 7methylguanosine triphosphate (GmTP)
 Following
is an INITIATION (leading) codon, AUG,
coding for methionine.
 A tail
at the end is made of many adenosine
monophosphates, called ‘poly-A tail’
 Codon:
Three monophosphate nucleotides that code for
a particular amino acid.

Cloverleaf structure with 4
arms: “hairpin loops”

tRNAs transfer the amino
acids to the ribosomal site
where protein synthesis is
taking place

At least 20 tRNAs for the 20
amino acids used in proteins

One arm has anticodon:
matches a codon on mRNA
(H-bonds to codon like
DNA… C=G , A=T)

Note: 3’ end has CCA which
carries a specific amino acid
(dictated by the codon)
E1: G6Pase
(von Gierke’s)
E2: PRPP
Synthetase
E8: DNase
E6: RNase
E4:
Ribonucleotide
reductase
E5: HGPTase
(Lesch-Nyhan)
E7: Xanthine
Oxidase
(hypouricemia)
E9: Adenosine
Deaminase
(SCID)
E9
E4
E1:Carbamoyl
Phosphate
Synthetase
E2: Aspartate
Transcarbamoylase
E3: PRPP
Synthetase
Inhibited
by CTP
Stimulated by PRPP
Inhibited by UTP
Inhibited by all
mono and diphosphate nts
Purine
Nitrogen base:
a. Amino acids: Gln, Asp, Gly
b. CO2
c. Coenzyme: Folate
Pyrimidine
Gln, Asp
NAD, NADPH
Folate
For Pentose Phosphate:
a. Pentose : R-5-P + ATP  PRPP
(via PRPP synthetase)
To form
a. Cofactor:
b. Energy:
IMP
Mg++
ATP
OMP
Mg++
ATP
Nucleotides and Nucleic Acids
3:00 to answer all eight
Answer choices for all questions:
a. DNA
b. mRNA
c. rRNA
d. tRNA
1. Thymine occurs in which one?
2. In humans, genes are segments of which one?
3. Which one is composed of codons and is directly responsible
for the amino acid sequence of a protein?
4. Which one possesses an anticodon and is responsible for the
transfer of amino acids to the site of protein synthesis?
5. In humans, the molecule of which one entirely exists as an
antiparallel double helix?
6. Which one has 7 methyl guanosine triphosphate at the 5’ end?
7. Which one possesses a polyA tail at the 3’ end?
8. Which one complexes with proteins to form ribosomes?
1:30 to answer all three
Answer choices for all questions:
a. Ribose-5-phosphate
b. 2’-deoxythymidine-5’-phosphate
c. Allopurinol
d. Uric Acid
9. Which one is a synthetic purine compound and could be
used to treat gout?
10. Which one is required for the biosynthesis of purine
nucleotides?
11. Which one is required for the biosynthesis of pyrimidine
nucleotides?
2:00 to answer all four
Answer choices for all questions:
a. Reverse transcriptase
b. Restriction endonuclease
c. Protein tyrosine kinase
d. Peptidyl transferase
12. Which one is responsible for the formation of the peptide bond
between amino acid residues in protein translation?
13. Which one is responsible for generating blunt or sticky ends of
DNA?
14. AZT, a drug used to treat AIDS patients, is an inhibitor of which
one?
15. The activity of which one is believed to initiate cell division
resulting in cancer?
Answer choices for all questions:
a. DNA
b. mRNA
c. rRNA
d. tRNA
1. Thymine occurs in which one?
A
2. In humans, genes are segments of which one?
A
3. Which one is composed of codons and is directly responsible
B for the amino acid sequence of a protein?
4. Which one possesses an anticodon and is responsible for the
D transfer of amino acids to the site of protein synthesis?
5. In humans, the molecule of which one entirely exists as an
A antiparallel double helix?
6. Which one has 7 methyl guanosine triphosphate at the 5’ end?
B
7. Which one possesses a polyA tail at the 3’ end?
B
8. Which one complexes with proteins to form ribosomes?
C
Answer choices for all questions:
a. Ribose-5-phosphate
b. 2’-deoxythymidine-5’-phosphate
c. Allopurinol
d. Uric Acid
9. Which one is a synthetic purine compound and could be
used to treat gout?
C
10. Which one is required for the biosynthesis of purine
nucleotides?
A
11. Which one is required for the biosynthesis of pyrimidine
nucleotides?
A
Answer choices for all questions:
a. Reverse transcriptase
b. Restriction endonuclease
c. Protein tyrosine kinase
d. Peptidyl transferase
12. Which one is responsible for the formation of the peptide bond
D between amino acid residues in protein translation?
13. Which one is responsible for generating blunt or sticky ends of
DNA?
B
14. AZT, a drug used to treat AIDS patients, is an inhibitor of which
one?
A
15. The activity of which one is believed to initiate cell division
resulting in cancer?
C
Characteristics of vitamins:
Cannot be synthesized by mammalian cells and,
therefore, must be supplied in the diet.
Exceptions: made by humans
Vitamin D3 –Fat soluble vitamin made in skin
Vitamin B3 (niacin) –Water soluble made in cells
Exceptions: made by bacteria in our gut
Vitamin K –Fat soluble
Vitamin B7 (biotin) –Water soluble
Fat soluble
Water soluble
Solubility
Good in fat/oil and
organics
Good in water
Absorption
Increased by fatty food
By intestinal capillaries
(except B12)
Transport
By specific proteins or
lipoproteins
By plasma, except B12
and folate
Storage
Significant. Mostly liver
and adipose
Lower. Mostly internal
organs and muscle
Function
Varies
Mostly as coenzymes and
antioxidants
Active site
Cellular lipid
components / nucleus
Aqueous cellular
components
Excretion
Slow, via bile
Fast; via urine
Deficiency
Presents slowly
Presents rapidly
Toxicity
high, except Vit E
Relatively low, except B3
Glycogen phosphorylase
Glycogen
B6
Glyceraldehyde 3-P
Glyceraldehyde 3-P DH
1,3 Bisphosphoglycerate
B3
B1 B2
B3 B5
B7
Pyruvate carboxylase
B2
B5
fumarate
Succinate DH
isocitrate B3
isocitrate DH
B6
B1 B3
Matching:
B1
B2
B3
B5
B6
B12
Vitamin C
pantothenic acid
cobalamin
riboflavin
ascorbic acid
niacin
thiamine
pyridoxine
Disease
MC S/S or distinguishing S/S
B1
Beriberi (deficiency)
Wet- edema
B2
Riboflavin deficiency
Dermatitis, cheilosis, lacrimation
B3
Pellagra (deficiency)
3 Ds: dematitis, diarrhea, dementia;
Casal’s necklace
B5
Pantothenic acid deficiency
rare
B6
Pyridoxine deficiency
converts Trp to niacin
Iron-loading microcytic anemia;
Pellagra if no B3 made
B7
Biotin deficiency (carboxylase rxns) Antibiotics / sulfonamides; seborrheic
dermatitis
B9
Folate deficiency
Neural tube defects; megaloblasticmacrocytic anemia
B12
Pernicious anemia (deficiency) –
requires Castle’s intrinsic factor
megaloblastic macrocytic anemia
Beriberi
Pernicious anemia –due
to lack of Castle’s
Intrinsic Factor
Iron-loading microcytic
anemia
Scurvy
Pellagra
Megaloblasticmacrocytic anemia
Thiamin deficiency
Riboflavin deficiency
Niacin deficiency
Pantothenic acid
deficiency
Pyridoxine deficiency
Biotin deficiency
Folate deficiency
Cobalamin deficiency
Vitamin C deficiency
Adult “wet” beriberi
Adult “dry” beriberi
Scurvy
Pellagra
Pernicious anemia
Gingivitis, swollen joints
& aching bones, delayed
wound healing, muscle
cramps, lassitude, dry and
scaly skin
Polyneuropathy, eye
rigidity w/ nystagmus,
muscle weakness, die of
infection, no edema
Due to lack of Castle’s
Intrinsic Factor
Edema, breathing
difficulty, tachycardia,
congestive heart failure
Dermatitis, Diarrhea,
Dementia
Gingival hemorrhage
bowed legs, stunted bone
growth, and swollen joints.
Infants fed only cow's milk are at
risk of developing scurvy, since
cow's milk is not an adequate
source
of vitamin C.
periungual
hemorrhage
Typical rash
 AI
for newborns: 40 mg/day
 RDA


75 mg/day for females
90 mg/day for males
 UL

for adults:
for adults: 2 g/day
A daily intake of 200-300 mg would saturate the blood
(1.2-1.5 mg/dL)

Excess excreted in urine
Anti-pernicious anemia factor
FMN
Pyridoxamine
Vitamin B1
Vitamin B2
Riboflavin
Vitamin B3
Nicotinic acid
Vitamin B5
TPP
Vitamin B6
CoASH
Methylcobalamin
Vitamin B7
Biotin
Vitamin B9
Anti-pellagra factor
Vitamin B12
Thiamin
Vitamin C
Pantothenic acid
H4-folate
Niacinamide
Vitamin B1
Vitamin B2
Dehydroascorbic acid
Vitamin B3
5’-deoxyadenosylcobalamin
Vitamin B5
FAD
Vitamin B6
NADP
Vitamin B7
Anti-beriberi factor
Pantetheine
Vitamin B9
Folacin
Vitamin B12
Pyridoxal phosphate
Vitamin C
Most important functions
Vitamin A: Synthesis of rhodopsin, epithelial cell
differentiation, bone growth and
wound healing
Vitamin D: Regulation of calcium levels
(bone and blood)
Vitamin E: Antioxidant
Vitamin K: Activates blood-clotting factors

Pro-vitamin A
Carotenoids: α-carotene, β-carotene, γcarotene
Most active
Vitamin A vitamers

Retinol, Retinal, Retinyl ester, Retinoic acid
Non-vitamin A carotenoids

Some carotenoids are not converted to
vitamin A

Drug form
13 cis-retinoic acid / all-trans retinoic acid
(Accutane or Retin A)
Retinol is stored in the liver as retinyl esters.
If Question about retina (night blindness, etc):
All trans-retinal  11-cis retinal
If Question about differentiation, choose retinoic acid
Uses: Dermatology, leukemia. It is a teratogen
Food sources:
Pro-vitamin A – think yellow/orange foods
Retinyl ester (MC vitamer) – liver, fish, dairy eggs
Mobilization:
Retinol is bound to Retinol Binding Protein in the plasma
Zn2+ is required for synthesis of RBP
and for retinol binding RBP

Symptoms:

Eye lesions: Nyctalopia, xerophthalmia, Bitot’s spot,
keratomalacia  permanent blindness

Epithelial disorders: Keratinization (toad skin), follicular
hyperkeratosis, infections of eyes, ears, respiratory tract

Reproductive failure

Digestion: Diarrhea, decrease in appetite
Pro-vitamin D  Pre-vitamin D  Vitamin D
(2-step process in order to be usable)
1. UV-dependent: produces pre-form
2. Temperature dependent: makes vitamins D2 or D3
Equally active in humans
Ergosterol  Pre-D2  Ergocalciferol (vit D2)



Occurs in yeast and some plants
Ergo: fungus/mushrooms.
Vitamin D2 is the form supplemented in MILK
7-dehydrocholesterol Pre-D3 cholecalciferol (vit D3)


Occurs in human subcutaneous tissue
Made from cholesterol
Food Sources
Fish, Egg yolk,
Butter (fat/oil),
Fortified foods:
Milk, margarine,
breakfast cereals,
pastries, breads.
Seasonal Vitamin D
deficiency has
been reported for
people living in
cold regions during
the winter
Humans can make
only one fat
soluble vitamin:
Vitamin D3

Like all fat-soluble vitamins, Vitamin D is
absorbed in the small intestine, bound to
chylomicrons, and transported through the lymph.

Endogenously synthesized vitamin D is bound to
α-2-globulin (plasma protein) for transport

Vitamin D is primarily stored in the liver.

Mobilization of vitamin D, and conversion to its
active form, occurs only when needed and is
under hormonal regulation

Vitamins D2 and D3 are stored in the liver as 25-hydroxy(chole/ergo)calciferol
cholecalciferol
hydroxylation
25-hydroxy-cholecalciferol
hydroxylation
Biochemically
Active form
1,25-dihydroxy-cholecalciferol

Effect of Vit D3 on Intestine and Kidney:
↑ synthesis of Calcium Binding Protein (calbindin)
causing re-absorption of Ca and P from urine
…To maintain serum calcium at optimal levels
(~5 mM or 10 mg/dL)

Effect of Vit D3 on Bone:
Low serum calcium triggers PTH release. PTH
stimulates 1-hydroxylase to form more 1,25(OH)2D,
which at high concentration causes bone resorption
 releases Ca2+ to increase serum Ca2+
parathyroid
1,25(OH)2D3
kidney

Self-regulation: (negative feedback): High 1,25(OH)2D
stimulates 24-hydroxylase to make more active
24,25(OH)2D, instead of 1,25(OH)2D.
parathyroid
1,25(OH)2D3
1,25(OH)2D2D
24,25(OH)
1,25(OH)23D23D
24,25(OH)
1,25(OH)23D23D
kidney 24,25(OH)
1,25(OH)23D23D
24,25(OH)
24,25(OH)323D3
1. The only fat-soluble vitamin made by humans
2. Absorbed in sm. intestine  chylomicron  lymph 
blood  liver (stored as 25-hydroxy D3)
3. Activated in liver by 1-hydroxylase (PTH)
Inhibited by 24-hydroxylase (calcitonin)
4. Maintains serum calcium levels
(reabsorbs from urine and takes from bone)

Symptoms:

Rickets in children: Bow legs, knock
knees, rachitic rosary of ribs, pigeon
chest, narrow pelvis, spinal curvature,
skull deformation, muscle weakness,
irritability, tetanus convulsion, poor
teeth

Osteomalacia in adults: Bow legs, bone
fractures, pain in the bone of legs and
lower back, walking difficulty, muscle
weakness

Also: Rachitic rosary

“Hypervitaminosis D”  hypercalcemia

Signs/symptoms:

Anorexia, Nausea, Vomiting
Polyuria, Polydipsia
Weakness, Nervousness
Renal failure
Calcification of soft tissues
(joints, lung, kidneys, blood vessels,
heart… can be fatal)




Calcium comes from bone

Food Sources: Fats and oils
Vegetable oils: safflower, sunflower, soybean
Dairy: Whole milk (in the fat)
Meat / Fish: liver
Grains and Fortified food: margarine

Main Function
Antioxidant: Neutralizes free radicals. Preserves
membrane integrity, and fat soluble factors
 Found primarily in mitochondria.

Absorption
Passive absorption (no protein carrier) in the sm intestine
 Transported by chylomicron via lymph to the liver


Transport
First absorbed inside a chylomicron  Packaged into
HDLs, LDLs, and VLDLs
 α-tocopherol is selectively incorporated into VLDL, due
to its high affinity for α-TTP (α-tocopherol transfer
protein), accounting for its high biological activity.


Storage



Constituent of the membrane
Primarily stored in adipose tissue
Mobilized and transported by LDL, HDL and VLDL
Deficiency is rare
 abundant in foods and well stored in the body

Symptoms:
 Neurological:
Areflexia, Cerebellar ataxia
 Loss of position sense, Loss of vibration sense,
 Muscle weakness

Excess

Prolonged bleeding time (anti vitamin K)
 May compete with vitamin A and D


Vitamin E competes with the other fat-soluble vitamins



Vitamin K is absorbed in the small intestine  bound
to chylomicrons and transported through lymph.
Excessive intake of Ca2+ impairs vitamin K absorption
Storage of vitamin K in the body is relatively low


Perhaps due to continuous supply by the intestinal bacteria.
Major storage is in liver

Other sites: skin, muscle, kidney and heart
Blood coagulation

Converts blood coagulation factors II, VII, IX, X from
pre-forms to pro-forms
Prevents excessive blood coagulation

Converts pre-C and pre-S proteins to C and S
proteins… which destroy factors V and VIII when
levels get too high
Stimulates mineralization of bone and teeth

γ carboxylation of osteocalcin (binds Ca2+ to bone &
teeth)
Maintains serum calcium levels

by working with PTH to increase kidney re-absorption
of Ca2+; the γ carboxylation of kidney glutamic protein
(KGP) is stimulated by PTH


Primary causes


poor placental transfer from mother
Lack of intestinal flora in newborn

Hospital practice: inject 1 mg of K1 or synthetic K3 upon birth
Secondary causes



Use of antibiotics (may kill intestinal flora)
Excessive anticoagulants (dicumarol, warfarin)
Symptoms of deficiency:




Impaired blood coagulation
Prolonged bleeding
Muscular and subcutaneous hemorrhage
Impaired mineralization of bone and teeth






Lipid foods increase fat-soluble vitamin absorption
Vitamin A increases the number of receptors for
1,25(OH)2D, and enhances the action of vitamin D
Adequate intake of protein and Zinc are essential
for the mobilization of stored Vitamin A
Mineralization of bone and teeth requires the
simultaneous adequate intake of Vitamins A and D,
Ca, P, Mg, Cl
Vitamin E and Se are both anti-oxidants and
enhance each other’s activity; they also spare
vitamin A.
Excessive intake of vitamin E antagonizes vitamins
A, D, and K
Vitamins
2:30 to answer all four
3. A deficient intake of vitamin __
1. Which vitamin is NOT
can develop pellagra because
involved in lowering the
blood level of homocysteine
___ is not converted to ____
a. Pyridoxine
a. B6, glutamate, glutamine
b. Riboflavin
b. B2, riboflavin, FAD
c. Folic acid
c. B6, tryptophan, niacin
d. Cobalamin
d. B12, dUMP, dTMP
2. A patient with photophobia, 4. The “Methyl Folate Trap”, a
lacrimation and cheilosis is
condition of accumulation of
most likely deficient in
excessive methyl folate, is
vitamin ___
caused by the deficiency of ___
a. Thiamin
a. Cobalamin
b. Riboflavin
b. Folic acid
c. Niacin
d. Biotin
c. Thiamin
d. Niacin
2:00 to answer all four
7. A patient who has red, swollen
5. Glycolysis cannot occur
gums and complains of bone
without the coenzyme form of
vitamin ______
pain may be deficient in
a. Thiamin
a. Vitamin B6
b. Riboflavin
b. Cobalamin
c. Niacin
c. Folacin
d. Biotin
d. Ascorbic acid
6. Glutathione reductase is a
flavoprotein because it
possesses vitamin ____ in its
structure
a. B1
b. B2
c. B3
d. B6
8. Coenzyme A, which is needed
for the metabolism of lipids is
derived from
a. Pantothenic acid
b. Cobalamin
c. Folacin
d. Ascorbic acid
1:30 to answer all three
9. The primary cause of pernicious anemia is due to the
deficiency of
a. Vitamin B6
b. Cobalamin
c. Folic acid
d. Castle’s intrinsic factor
10. A patient with megaloblastic-macrocytic anemia may be
deficient in
a. Vitamin B1 or B2
b. Cobalamin of folic acid
c. Vitamin B5 or B6
d. Vit C or Vitamin B6
11. Even with adequate dietary intake, alcoholics are most likely
to be deficient in
a. Vitamin A
b. B vitamins
c. Vitamin C
d. Vitamin D
1:00 to answer both
12. The reason vitamin C is a good anti-oxidant is
because
a. It is a strong oxidant
b. It is a strong reductant
c. It is a strong acid
d. It is a strong base
13. The coenzyme form of which one is NOT required
for the conversion of pyruvate to acetyl-CoA in the
use of glucose for energy
a. Thiamine
b. Riboflavin
c. Niacin
d. Biotin
2:30 to answer all four
16. Infantile desquamative
14. A man who complains of
erythroderma can be
numbness and tingling in his
extremities, weakness and mental
caused by deficiency of
confusion might be suffering
a. Vitamin B1
from a deficiency of
b. Vitamin B2
a. Vitamin B1
c. Vitamin B3
b. Vitamin B2
c. Vitamin B3
d. Biotin
d. Biotin
17. The coenzyme from of
15. Which vitamin listed is LEAST
which one is not required
likely to be related to health of
for the Kreb’s cycle
skin and other epithelial tissues
a. Vitamin A
a. Vitamin B5
b. B Vitamins
b. Vitamin B2
c. Vitamin C
c. Vitamin B3
d. Vitamin D
d. Biotin
1:30 to answer all four
Answer choices for all questions:
a. FAD / FADH2
b. NAD+ / NADH + H+
c. CoASH
d. Pyridoxal phosphate
18. Which one is derived from pantothenic acid?
19. Which one is derived from vitamin B6?
20. Which one is derived from riboflavin?
21. Which one is derived from nicotinamide?
1:00 to answer both
22. Thiamine pyrophosphate is the coenzyme form of which one
a. Vitamin B1
b. Vitamin B2
c. Niacin
d. Vitamin B6
23. The daily requirement of which one is proportional to the daily
protein need because it is essential for amino acid metabolism
a. Pantothenate
b. Pyridoxine
c. Cobalamin
d. Folate
1. Which vitamin is NOT
involved in lowering the
blood level of homocysteine
a. Pyridoxine
b. Riboflavin
c. Folic acid
d. Cobalamin
3. A deficient intake of vitamin __
can develop pellagra because
___ is not converted to ____
a. B6, glutamate, glutamine
b. B2, riboflavin, FAD
c. B6, tryptophan, niacin
d. B12, dUMP, dTMP
2. A patient with photophobia, 4. The “Methyl Folate Trap”, a
lacrimation and cheilosis is
condition of accumulation of
most likely deficient in
excessive methyl folate, is
vitamin ___
caused by the deficiency of ___
a. Thiamin
a. Cobalamin
b. Riboflavin
b. Folic acid
c. Niacin
d. Biotin
c. Thiamin
d. Niacin
5. Glycolysis cannot occur
without the coenzyme form of
vitamin ______
a. Thiamin
b. Riboflavin
c. Niacin
d. Biotin
7. A patient who has red, swollen
gums and complains of bone
pain may be deficient in
a. Vitamin B6
b. Cobalamin
c. Folacin
d. Ascorbic acid
6. Glutathione reductase is a
flavoprotein because it
possesses vitamin ____ in its
structure
a. B1
b. B2
c. B3
d. B6
8. Coenzyme A, which is needed
for the metabolism of lipids is
derived from
a. Pantothenic acid
b. Cobalamin
c. Folacin
d. Ascorbic acid
9. The primary cause of pernicious anemia is due to the
deficiency of
a. Vitamin B6
b. Cobalamin
c. Folic acid
d. Castle’s intrinsic factor
10. A patient with megaloblastic-macrocytic anemia may be
deficient in
a. Vitamin B1 or B2
b. Cobalamin of folic acid
c. Vitamin B5 or B6
d. Vit C or Vitamin B6
11. Even with adequate dietary intake, alcoholics are most likely
to be deficient in
a. Vitamin A
b. B vitamins
c. Vitamin C
d. Vitamin D
12. The reason vitamin C is a good anti-oxidant is because
a. It is a strong oxidant
b. It is a strong reductant
c. It is a strong acid
d. It is a strong base
13. The coenzyme form of which one is NOT required for
the conversion of pyruvate to acetyl-CoA in the use of
D-glucose for energy
a. Thiamine
b. Riboflavin
c. Niacin
d. Biotin
16. Infantile desquamative
14. A man who complains of
erythroderma can be
numbness and tingling in his
extremities, weakness and mental
caused by deficiency of
confusion might be suffering
a. Vitamin B1
from a deficiency of
b. Vitamin B2
a. Vitamin B1
c. Vitamin B3
b. Vitamin B2
c. Vitamin B3
d. Biotin
d. Biotin
17. The coenzyme from of
15. Which vitamin listed is LEAST
which one is not required
likely to be related to health of
for the Kreb’s cycle
skin and other epithelial tissues
a. Vitamin A
a. Vitamin B5
b. B Vitamins
b. Vitamin B2
c. Vitamin C
c. Vitamin B3
d. Vitamin D
d. Biotin
Answer choices for all questions:
a. FAD / FADH2
b. NAD+ / NADH + H+
c. CoASH
d. Pyridoxal phosphate
18.
C
19.
D
20.
A
21.
B
Which one is derived from pantothenic acid?
Which one is derived from vitamin B6?
Which one is derived from riboflavin?
Which one is derived from nicotinamide?
22. Thiamine pyrophosphate is the coenzyme form of which one
a. Vitamin B1
b. Vitamin B2
c. Niacin
d. Vitamin B6
23. The daily requirement of which one is proportional to the daily
protein need because it is essential for amino acid metabolism
a. Pantothenate
b. Pyridoxine
c. Cobalamin
d. Folate
 Factors

Physiologic needs





increasing absorption
Pregnancy, lactation
Growth
Phlebotomy
Menstruation, chronic bleeding
Iron form (e.g. heme, Fe3+)

Ascorbic acid (vitamin C) and low pH enhance absorption by
favoring Fe2+ over Fe3+

Best absorbed as heme, as found in hemoglobin and
myoglobin
 Newborn
0.27 mg/day
 Adult
males
8 mg/day
 Adult
females 19-50
18 mg/day
 Adult
females 51+
8 mg/day
 Pregnancy
27 mg/day
 AI

Adults: 3.8 grams of table salt (1 tsp)


1.5 g of Na
2.3 g Cl
 UL:
5.8 grams of table salt
 People consume salt in excess of UL
Mineral
Function
Calcium
Hydroxyapatite; cellular regulation
Phosphorus
Hydroxyapatite; osmotic balance; ATP, phospholipids, and
other phosphate-containing molecules
Magnesium
Enzyme cofactor or activator, especially for energy
extraction (think ATP) and RNA related enzymes
Sodium
Electrolyte for nerve and muscle; glucose absorption;
regulates blood pressure
Potassium
Potassium Electrolyte for nerve and muscle; protein
synthesis; metabolism of carbohydrate (pyruvate kinase);
raise pH; reduce bone loss
Chloride
Gastric acid formation; cofactor for α-amylase
Sulfur
Amino acid function (part of Cys & Met); Vitamin function
(component of thiamine, biotin and pantothenic acid);
Structure of connective tissue (component of some GAGs
e.g. heparin); Bile function (taurocholic acid)
Iron
Hemoglobin, myoglobin, heme-containing enzymes, nonheme iron-containing enzyme
Mineral
Function
Copper
Ceruloplasmin component, e.g., iron uptake in red
marrow; Connective tissue development through
collagen cross linking
Zinc
Cofactor and component of metalloproteins… think
vitamin A and insulin
Iodine
Thyroid hormones (T4 and T3) and BMR setting
Manganese
Sphingosine synthesis; Carb metabolism, superoxide
dismutase
Fluoride
Hard tissue mineral, (fluoroapatite), anti-cariogenic
(cavity)
Molybdenum
Uric acid
Selenium
Antioxidant / enzyme component (glutathione
peroxidase)
Chromium
Increased insulin function; Increases HDL, lowers LDL
Cobalt
Vitamin B12 synthesis
Boron
Role in bone
DEFICIENCY
Excess
Fe
↑TIBC & ↑transferrin
Chronic fatigue, headache, pallor,
infection, Depression
↑transferrin
Hemosiderosis
Hemochromatosis
Cu
Menke’s dz:
Steely hair, diarrhea, albinism,
nerve degeneration
Wilson’s dz:
MR, tremor, Kayser-Fleischer ring
Zn
Acrodermatitis enteropathica:
(Genetic) ↓absorporption
Vomit, diarrhea, abdominal
cramps
I
Simple goiter, cretinism, deafmute, motor rigidity
Iodide goiter, Hyperactivity
F
Tooth decay
Fluorosis: Mottled teeth, black
bone
Se
Keshan dz: white nail beds,
Anemia
Cirrhosis, splenomegaly, GI
bleeding, depression, dental caries
Minerals
45 sec to answer both
1. Which nutritional abnormality is NOT associated with
hypertension
a. Excess of sodium
b. Sodium deficiency
c. Deficiency of potassium
d. Deficiency of calcium
2. The deficiency of which one would NOT impair the body’s
ability to remove superoxide
a. Copper
b. Zinc c. Manganese d. Fluoride
45 sec to answer both
3. Thyroid goiter could be caused by
a. Iodine deficiency
b. Iodine excess
c. Manganese deficiency
d. Both ‘a’ and ‘b’
4. Which is NOT known to be critical to bone
a. Calcium
b. Phosphate
c. Boron
d. Potassium
1:00 to answer all three
5. The ionic form of which one is a common cofactor for
kinases
a. Calcium
b. Magnesium
c. Sodium
d. Potassium
6. Hemosiderosis is due to the toxicity of which one
a. Iron
b. Copper
c. Zinc
d. Iodine
7. Dark brown chalk-like powder on the surface of teeth could
be due to toxicity
a. F
b. Se
c. Cr
d. B
1:30 to answer all three
8. Which of the following is toxic to the electron transport
chain, but a trace amount is also needed by humans
a. Potassium
b. Lead
c. Arsenic
d. Aluminum
9. Excessive intake of which one is teratogenic in birds and
humans, and increases the risk of mental retardation and
learning disability
a. Na
b. K
c. Se d. Ca
10. The organic form of Se is selenomethionine which occurs
in_____
a. Glutathione reductase
b. Glutathione peroxidase
c. Glutathione transhydrogenase
d. Glutathione transsulfurase
1. Which nutritional abnormality is NOT associated with
hypertension
a. Excess of sodium
b. Sodium deficiency
c. Deficiency of potassium
d. Deficiency of calcium
2. The deficiency of which one would NOT impair the body’s
ability to remove superoxide
a. Copper
b. Zinc c. Manganese d. Fluoride
3. Thyroid goiter could be caused by
a. Iodine deficiency
b. Iodine excess
c. Manganese deficiency
d. Both ‘a’ and ‘b’
4. Which is NOT known to be critical to bone
a. Calcium
b. Phosphate
c. Boron
d. Potassium
5. The ionic form of which one is a common cofactor for
kinases
a. Calcium
b. Magnesium
c. Sodium
d. Potassium
6. Hemosiderosis is due to the toxicity of which one
a. Iron
b. Copper
c. Zinc
d. Iodine
7. Dark brown chalk-like powder on the surface of teeth could
be due to toxicity
a. F
b. Se
c. Cr
d. B
8. Which of the following is toxic to the electron transport
chain, but a trace amount is also needed by humans
a. Potassium
b. Lead
c. Arsenic
d. Aluminum
9. Excessive intake of which one is teratogenic in birds and
humans, and increases the risk of mental retardation and
learning disability
a. Na
b. K
c. Se d. Ca
10. The organic form of Se is selenomethionine which occurs
in_____
a. Glutathione reductase
b. Glutathione peroxidase
c. Glutathione transhydrogenase
d. Glutathione transsulfurasea
1. Adrenaline (catecholamine made from tyrosine)
What you should know:
If a pathway produces energy (or molecules used to
produce energy), epinephrine stimulates it
If a pathway stores energy, epinephrine inhibits it
2. Thyroxin: (also from tyrosine)
Increases the BMR by elevating enzyme activity
Liver and muscle mostly affected
1. Pituitary hormones:
Anterior: GH, prolactin, TSH, ACTH, FSH, LH
Posterior: oxytocin and ADH
2. Insulin:
If a pathway stores energy or molecules used for energy,
insulin stimulates it
It inhibits Hormone-sensitive lipase
Diabetes mellitus due to insufficient insulin
(Type I) or non-functional insulin receptors
(Type II)
3. Glucagon: opposes insulin
Stimulates glycogenolysis (same as epinephrine) but does
not increase glycolysis or stimulate Σ nervous system
 therefore glucose is released to blood
4. Somatostatin (secreted by hypothalamus):
It inhibits secretion of GH, TSH, ACTH, cholecystokinin,
insulin and glucagon
Although formed in different tissues due to different sets
of enzymes, all are formed from cholesterol

Cholesterol is mainly derived from blood plasma, some is
from intracellular synthesis

For all steroid hormones, the first enzyme is cholesterol
desmolase (P-450scc) which cleaves the side-chain of
cholesterol (missing this enzyme is fatal)

All steroid hormones are produced by adrenal cortex
Gonado-corticoids are also produced by sex organs
How all steroid hormones work
They bind receptors  nucleus  binds DNA 
alters rate of RNA synthesis  affects amount of
proteins / enzymes
Glucocorticoids
One important physiological function is the
stimulation of gluconeogenesis from proteins.
Examples: cortisol (hydrocortisone) and
corticosterone
Synthesized in the adrenal cortex.
Effect on carbohydrates

Gluconeogenesis (Insulin opposes)


Induce liver enzymes involved in use of amino acids
for gluconeogenesis
Glycogenesis

Increases protein phosphatase (which converts
glycogen synthase from b-form to a-form)
Effect on lipids
Lipolysis
Lipogenesis
In extremities
In face and trunk
Effect on protein
Hepatic protein synthesis
Extrahepatic catabolism
Presumably to provide
more amino acids for
gluconeogenesis
Cushing’s Syndrome:
Glucocorticoid excess due to
excessive pharmacologic
doses, adrenal carcinoma,
excessive ACTH from
pituitary adenoma
Mineralocorticoids
Enhance Na+ retention
enhance excretion of K+, H+, NH4+.
The primary endogenous mineralocorticoid is
aldosterone
Conn’s Syndrome: Primary aldosteronism
Raised aldosterone levels due to adenoma of
glomerulosa cells (synthesizing aldosterone)
Addison’s Disease:
Primary adrenal insufficiency
Generalized skin pigmentation (white patient)
Gums, nails, palmer creases
Secondary Adrenal Insufficiency:
Due to ACTH deficiency
Adrenal hormones
Cortisol and corticosterone  glucocorticoids
Aldosterone  mineralocorticoid
Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)
A group of conditions affecting cortisol production. Often
involve greater or lesser production of sex steroids and can alter
development sex characteristics in affected people
A small minority of people can be said to be hermaphrodites
Deficiency of P-450scc (cholesterol desmolase)
Deficiency can be fatal
Reproductive Steroids (gonadocorticoids)

Androgens (Male sex steroids)
Primarily produced by testes. Examples:
testosterone and dihydrotestosterone (DHT).
Testes also make some 17β-estradiol (estrogen)

Estrogens (Female sex steroids)
Primarily produced by ovaries. Examples: 17βestradiol (estrogen) and estrone

Progestins (Gestational steroids)
Produced by corpus luteum and placenta.
Example: Progesterone
Biochemical effects




Sexual differentiation
Spermatogenesis
Anabolic metabolism
Male-pattern behavior
Biosynthesis



Conversion of testosterone to DHT can occur in testes and
other tissues (e.g. muscle).
Conversion is a NADPH-dependent reduction by
5-α-reductase.
DHT is the most active androgen
 Biochemical
effects
Maturation of primordial germ cells
 Develop uterine tissues for implantation of blastocyst
 Regulation of ovulation
 Establish condition of pregnancy (w/ placental hormones)
 Effect of parturation and lactation

 Biosynthesis

Synthesized from androgens by the action of aromatase.
 Insulin


opposes Glucagon/Epinephrine
Increases entry of glucose and neutral amino acids into
target cells
Increases protein synthesis in skeletal muscle
Increases glycolysis (activates glucokinase, PFK-1)
 Decrease gluconeogenesis (inactivates PEPCK gene)





Increases glycogenesis (activates glycogen synthase)
Decreases glycogenolysis (inactivates glycogen
phosphorylase)
Increases lipogenesis (activates acetyl-CoA carboxylase)
Decreases lipolysis (inactivates hormone-sensitive lipase)
 IgG
antibody stimulates the thyroid
 Activates the TSH receptor
 Diffuse hypertrophy of the thyroid gland
 Uncontrollable production of T3 and T4
 In
response, the body decreases TSH
Hormones
2:00 to answer all four
Answer choices for all questions:
a. Niemann-Pick disease
b. Multiple sclerosis
c. Addison’s Disease
d. Conn’s Syndrome
1. Which one is associated with the deficiency of phospholipids
and sphingolipids in white matter?
2. Which one is due to the deficiency of sphingomyelinase, an
enzyme responsible for the catabolism of sphingomyelins?
3. Hypernatremia and hypokalemia are associated with which
one because of the excessive synthesis of aldosterone?
4. Impaired gluconeogenesis or hypoglycemia occur in which
one?
1:00 to answer all four
Answer choices for all questions:
a. Cortisol
b. Androgen
c. Estrogen
d. Progestin
5. Dihydrotestosterone is the most potent form of which one?
6. Which one is an aromatic steroid?
7. Anabolic steroids are analogues of which one?
8. Which one can increase the liver levels of alanine
aminotransferase and PEP carboxykinase?
1:30 to answer all three
Answer choices for all questions:
a. Desaturase
b. PEP-carboxykinase
c. Glycogen synthase
d. Aromatase
9. Glucocorticoids can stimulate gluconeogenesis by increasing
the liver of which one?
10. Which one is abundant in ovaries and is responsible for the
conversion of androgens to estrogens?
11. Which one catalyzes the synthesis of certain unsaturated
fatty acids from saturated fatty acids?
1:30 to answer all three
Answer choices for all questions:
a. Cortisol
b. Aldosterone
c. Dihydrotestosterone
d. 17-β-estradiol
12. Conn’s Syndrome is characterized by hypernatremia,
hypertension and hypokalemia, and is due to excessive
production of which one?
13. Anabolic steroids are structural analogues of which one?
14. Which one has the strongest anti-inflammatory action?
1:30 to answer all three
Answer choices for all questions:
a. Acetyl-CoA carboxylase
b. Carnitine-palmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
15. Insulin increases the activity of which one?
16. Epinephrine & glucagon suppress the activity of which one?
17. A high body level of cholesterol reduces the activity of
which one, (a way to regulate body cholesterol level)?
Answer choices for all questions:
a. Niemann-Pick disease
b. Multiple sclerosis
c. Addison’s Disease
d. Conn’s Syndrome
1. Which one is associated with the deficiency of phospholipids and
sphingolipids in white matter?
B
2. Which one is due to the deficiency of sphingomyelinase, an enzyme
responsible for the catabolism of sphingomyelins?
A
3. Hypernatremia and hypokalemia are associated with which one
because of the excessive synthesis of aldosterone?
D
4. Impaired gluconeogenesis or hypoglycemia occur in which one?
C
Answer choices for all questions:
a. Cortisol
b. Androgen
c. Estrogen
d. Progestin
5. Dihydrotestosterone is the most potent form of which one?
B
6. Which one is an aromatic steroid?
C
7. Anabolic steroids are analogues of which one?
B
8. Which one can increase the liver levels of alanine
A aminotransferase and PEP carboxykinase?
Answer choices for all questions:
a. Desaturase
b. PEP-carboxykinase
c. Glycogen synthase
d. Aromatase
9. Glucocorticoids can stimulate gluconeogenesis by increasing
the liver of which one?
B
10. Which one is abundant in ovaries and is responsible for the
conversion of androgens to estrogens?
D
11. Which one catalyzes the synthesis of certain unsaturated
fatty acids from saturated fatty acids?
A
Answer choices for all questions:
a. Cortisol
b. Aldosterone
c. Dihydrotestosterone
d. 17-β-estradiol
12. Conn’s Syndrome is characterized by hypernatremia,
B
hypertension and hypokalemia, and is due to excessive
production of which one?
C 13. Anabolic steroids are structural analogues of which one?
A 14. Which one has the strongest anti-inflammatory action?
Answer choices for all questions:
a. Acetyl-CoA carboxylase
b. Carnitine-palmitoyltransferase I
c. HMG-CoA reductase
d. Desaturase
15. Insulin increases the activity of which one?
A
16. Epinephrine & glucagon suppress the activity of which one?
A
17. A high body level of cholesterol reduces the activity of
C which one, (a way to regulate body cholesterol level)?