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L-carnitine
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Page 1
Background
In early years of life there is higher energy consumption;
mostly from fatty acids.
mostly from fatty acids.
Son’kin and Tambovtseva 2012; Kostyak JC et al (2007)
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Energy Sources
• The main energy source are fatty acids
Burning (power
(kcal/g)
Brennwert
kcal/ gramm)
12
Erdöl
Petrol
Fett
Fat
8
KohlenCarbo
hydrat
hydrates
4
0
Protein
Proteins
•
Alkohol
Alcohol
B-oxidation is the process producing energy from fatty acids
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ATP
Fatty Acids Transport and Oxidation
• L-Carnitine is crucial and exclusive for fatty acid
metabolism/oxidation
• L-Carntine allows the transport of fatty acids into the mitochondria
and guaranties the cellular energy requirements.
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Detoxification of toxic metabolites
• L-Carnitine neutralizes toxic metabolic
derivatives
• Long chain fatty acids accumulate and
become cytotoxic by degrading cellular
membranes and inhibiting enzymes
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Methabolic Pathways
• Fatty acid oxidation is fundamental
part of metabolic pathways
Carbohydrates
Protein
Fatty Acids
• The metabolic pathways link the main vital
organs and tissues
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Carnitine deficiencies
• impairment of the vital organs and tissues
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Carnitine Deficiency Types
Primary Carnitine Deficiency
Is a rare genetic defect of the
Carnitine transporter OCTN2 on the
cell membrane
Secondary Carnitine Deficiency
Is a consequence of:
• genetic defects
• pathologies
• drugs treatment
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Primary Carnitine Deficiency
May occur from infancy to adolescence.
Early recognition and treatment with high dose oral L-Carnitine is
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life –saving.
Primary Carnitine Deficiency Treatment
 Primary Carnitine Deficiency is fatal/letal, unless promptly diagnosed and
immediately treated with L-Carnitine.
 All life long treatment with high strength L-carnitine supplementation is
essential
and resolve critical clinical situations:(eg. Normalization of cardiac
function).
L-Carnitine will need to be continued for all life!
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Secondary Carnitine Deficiency
 Genetic causes - Inborn errors of Metabolism• Organic acidemias – amino acid oxidation errors
• Fatty acid oxidation defects
 Acquired causes
• Drug-induced Carnitine deficiency
• Physiopathologically-induced Carnitine deficiency
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Organic Acidemias
Organic Acidemias are Induced by Amino Acid Oxidation Defects
Toxic organic acids
A disease with high concentration of toxic metabolites =ACIDS
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Fatty Acid Oxidation Defects
 Carnitine system enzyme defects
 Beta oxidation defects
 Mitochondrial respiratory chain enzyme defects
Toxic organic acids
Induce high concentration of toxic metabolites
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Drug-induced Carnitine deficiency
Valproic acid is a broad-spectrum antiepileptic
Valproic acid oxidation induces high concentration of toxic drug metabolites
and block the urea cycle – hyperammonemia!
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Secondary Carnitine Deficiency Treatment
Detoxification activity performed by L-carnitine
Toxic organic acids
L-carnitine chronic treatment increases the elimination of toxic metabolites
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Physiopathologically-induced Carnitine deficiency
Type 1 diabetes (T1DM)
Autoimmune destruction (apoptosis) of the insulin-producing pancreatic
beta cells.
Lack of insulin leads to increase of glucose in the blood and loss in the
urines.
•
Lower plasma levels of Total Carnitine and Free Carnitine
•
Echocardiographic and Metabolic parameters Worsening
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Carnitine treatment in T1DM
To Prevent Apoptosis
To Improve Cardiological and
Metabolic Parameters
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谢谢
INTERNATIONAL DIVISION
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