Download 3 path organels introduction

General Pathology
Pathology of
Organels
– Introduction
Jaroslava Dušková
The Secretory
Process
and
Its Disorders
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
http://www1.lf1.cuni.cz/~jdusk/
The Cell Components &Their Function
1.
nucleus (incl. nucleolus and
nuclear envelope)
2.
3.
cytoplasm

cytoplasmic matrix

cytoplasmic organelles
plasma membranes
The Cell Components &Their Function
The nucleus
(incl. nucleolus and nuclear
envelope)
chromosomes
DNA, RNA, histones
The Cell Components &Their Function
The nucleus functions
– cell division
– genetic information
transcription & control
Neoplasia (Tumour)

DNA disease

Stepwise accumulation
of genetic abnormalities
 Escape of immunological
clearing systems
The Cell Components &Their Function
The cytoplasm (cytosol)
cytoplasmic matrix
cytoplasmic organelles

ribosomes

peroxisomes

endoplasmic
reticulum

mitochondria

cytoskeleton

Golgi complex

caveolae

lysosomes

vaults
The Cell Components &Their Function
The cytoplasm (cytosol)
aqueous solution with enzymes
The Cell Components &Their Function
The cytoplasm function
– proteosynthesis
– storage unit
(in coop. with organelles)
(fat, carbohydrates and
secretory vesicles)
The Cell Components &Their Function
The cytoplasm - cytoplasmic organelles

ribosomes

peroxisomes

endoplasmic
reticulum

mitochondria

cytoskeleton

Golgi complex

caveolae

lysosomes

vaults
The Cytoplasmic Organelles Functions
(1)
Organelle
Composition
Function
ribosomes
RNA –protein
complexes
proteosynthesis
endoplasmic
reticulum
cisternae,tubular proteosynthesis
channels
& transport
Golgi complex smooth
(GC)
membranes &
vesicles
processing and
packaging
lysosomes
digestion
sacklike GC
derived
The Cytoplasmic Organelles Functions
Organelle
Composition
(2)
Function
peroxisomes lysosomes like detoxication
producing or
using H2O2
mitochondria membrane
bound energy
production
Oxydative
fosforylation, cell
signaling, pH
control, Ca
homeostasis
Mitochondrial DNA somatic mutations
(point mutations and large deletions)
and mtDNA variants in human thyroid
pathology
A study with emphasis on Hurthle cell
tumors
Máximo V et al, Am J Pathol 160:1857, 2002
Hürthle cell features
Nuclear genes
Ex. GRIM-19
Mt genes
(Complex I, III, IV, V)
Mitochondrial
proliferation
Activation of HIF-1
& Decreased
apoptosis
Tumourigenesis
Peroxisomes - microbodies
up to 2 microns - catalase
Function
Degradation: substrate oxidation
(etanol)
Anabolism: synthesis of
prostaglandin , cholesterol, billiary
acids, plasmalogens,
gluconeogenesis, transamination
The Cytoplasmic Organelles Functions
(3)
Organelle
Composition
Function
Cytoskeleton
Microtubules & actin Microvilli, cilia,
microfilaments
flagella
Caveolae
Membrane
indentations
Shuttling
material
Vaults
Octagonal barrrels
like
ribonucleoproteins
Shuttling
molecules
The Cell Components &Their Function
The plasma membranes
– cell surrounding
– organelles enclosing
– bilayer of lipids and proteins
Cell
mechanism
Membrane function
Structure
Compartmentalization, cytoskeleton & ER
contacts, fluid & electrolyte balance
Protection
Barrier to toxins & foreign organisms/cells
Activation of Hormones, mitogens, antigens, growth
cell
&proliferation factors
Storage
Receptors, transport, diffusion, exocytosis,
endocytosis
Cell to cell
interaction
Communication & attachment –junctional
complexes, nutritive relationship, enzymes
and antibody release
Organelles Involved in Secretion
 membrane
 maternal
type
origin (ovum cytoplasm)
 autoreplicative
– granular (rough) endoplasmic reticulum
– Golgi apparatus
– lysosomes
Exocytosis and Its Disorders
protein secretion on the granular
endoplasmic reticulum
cis Golgi network
trans Golgi network
(signal molecules
attachment)
Golgi Endoplasmic Reticulum
Lysosomes
Secretion
– exocrine
(apical pole of the cell
lumen, duct)
– endocrine (basal pole of the
cell
– paracrine
–
autocrine
blood)
influencing
neighbouring cells
self influencing
Secretion


continual - permanent unregulated
pulsatory - regulated
Secretion

continual - permanent
– unregulated vesicle transport
– protocolagen, proteoglycans, viral
particles
Targetting:
immunoglobulins without and
after stimulation
Secretion

pulsatory - regulated
– exocrine mucin or zymogen
–
–
–
–
–
–
–
granules secretion
endocrine cells
neurons
T-lymphocytes
heparinocytes
membrane budding
thrombocytes
coating proteins
granulocytes
endothelia
Secretion Disorders
defects of synthesis
product itself
 auxilliary proteins

retention in GER
(inborn endoplasmic reticulum storage
diseases)
hyaline droplets (Russel bodies)
Neuroendocrine Secretion

dense core granules

secretory vesicles
(small synaptic vesicles)
Neuroendocrine
Secretion Disorders
defects of synthesis
product itself
 auxilliary proteins

regulation disorders on the receptor
level
Membrane Components
of Secretory Granules and Vesicles
– Synaptophysin (synaptic vesicle protein)
– Neuron Specific Enolase
– S-100 protein
identification of neuroendocrine
neoplasms