Aluminum Caprate
Aluminum Palmitate
P-Aminobenzoic acid
Bebzakdegtde
Benzyl Acetoacetate
Benzyl Benzoate
Benzyl Butyrate
Benzyl Cinnamate
Benzyl Formate
Calcium Ascorbate
Calcium Bromate
Calcium Caprate
Calcium Caprylate
Calcium Carbonate
Calcium Citrate
Calcium Diglutamate
Calcium Gluconate
Calcium Lactate
Calcium Myristate
Calcium Palmitate
Calcium Pantothenate
D-Camphor
Carboxymethyl cellulose
Cellulose Acetate
Chlorophyll
Citronellal
DL-Citronellol
Citronellyl Acetates
Citronellyl Butyrate
Diethyl Malate
Diethyl Tartrate
Disodium Citriate
Erythorbic Acid
Ethyl Acetate
Neryl Acetate
Neryl Butyrate
Neryl Formate
Ethyl Vanillin
Ethtyl Vanillin Isobutyrate
Ethyl Vanillin Beta-D-Glucopyranoside
Ethyl Vanillin Propyleneglycol Acetal
Ferric Citrate
Ferrous Ascorbate
Ferrous Citrate
Ferrous Fumarate
Ferrous Lactate
Ferrous Gluconate
Geranyl Formate
Lactalbumin
Lanolin
Lauric Acid
Lauryl Acetate
Lauryl Alcohol
Linalyl Acetate
Linalyl Anthranilate
Linalyl Formate
Magnesium Caprate
Magnesium Caprylate
Magnesium Fumarate
Manganese Citrate
Musk, Ketone
Myristic Acid
Sodium Ascorbate
Sodium Benzoate
Sodium Bicarbonate
Sodium Formate
Sodium Fumarate
Sodium Humate
Sodium Lactate
Sodium Tartrate
Tocopherols
Sorbitol
Salicylic Acid
Potassium Sorbate
Potassium Chloride
Potassium Caseinate
Potassium Carbonate
Potassium Caprylate
Potassium Caprate
Beta-Carotene
Potassium Bromate
Potassium Borate
Potassium Bisulfite

 

L-Carnitine

(CAS: 461-06-3)

Chemical Name: (R)-3-Carboxy-2-hydroxy-N,N,N-trimethyl-1- propanaminium hydroxide, inner salt / (R)-(3-Carboxy-2-hydroxypropyl) trimethyl ammonium hydroxide, inner salt

Structure:

Characteristic:

L-Carnitine forms white crystals or a white crystalline hygroscopic powder. It has a slight characteristic odor.
L-Carnitine is extremely hygroscopic and even deliquescent and it can liquefy on exposure to air.
L-Carnitine melts above 200ˇćunder decomposition.
L-Carnitine is highly soluble in water, in alcohol, in alkaline solutions, and in dilute mineral acids. It is practically insoluble in acetone or ethyl acetate.

Application: L-Carnitine is a natural, vitamin-like nutrient responsible for promoting the ¦Â-oxidation process of long- chain fatty acids. In other words, it helps the body convert fatty acids into energy. L-Carnitine is often used in medicine and health food, sports drinks, infant nutrition and animal nutrition

Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and derived from meat and dairy products in the diet. It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA.

Carnitine deficiency is a metabolic state in which carnitine concentrations in plasma and tissues are less than the levels required for normal function of the organism. Biologic effects of low carnitine levels may not be clinically significant until they reach less than 10-20% of normal. Carnitine deficiency may be primary or secondary.

Primary carnitine deficiency is caused by a deficiency in the plasma membrane carnitine transporter, with urinary carnitine wasting causing systemic carnitine depletion. Intracellular carnitine deficiency impairs the entry of long-chain fatty acids into the mitochondrial matrix. Consequently, long-chain fatty acids are not available for beta-oxidation and energy production, and the production of ketone bodies (which are used by the brain) also is impaired.

Regulation of the intramitochondrial free CoA also is affected, with accumulation of acyl-CoA esters in the mitochondria. This, in turn, affects the pathways of intermediary metabolism that require CoA (eg, Krebs cycle, pyruvate oxidation, amino acid metabolism, mitochondrial and peroxisomal beta oxidation).

The 3 areas of involvement include (1) the cardiac muscle, which is affected by progressive cardiomyopathy (by far, the most common form of presentation), (2) the central nervous system, which is affected by encephalopathy caused by hypoketotic hypoglycemia, and (3) the skeletal muscle, which is affected by myopathy.

Muscle carnitine deficiency (restricted to muscle) is characterized by depletion of carnitine levels in muscle with normal serum concentrations. Evidence indicates that the causal factor is a defect in the muscle carnitine transporter.

In secondary carnitine deficiency, which is caused by other metabolic disorders (eg, fatty acid oxidation disorders, organic acidemias), carnitine depletion may be secondary to the formation of acylcarnitine adducts and the inhibition of carnitine transport in renal cells by acylcarnitines.

In disorders of fatty acid oxidation, excessive lipid accumulation occurs in muscle, heart, and liver, with cardiac and skeletal myopathy and hepatomegaly. Long-chain acylcarnitines also are toxic and may have an arrhythmogenic effect, causing sudden cardiac death.

Encephalopathy may be caused by the decreased availability of ketone bodies associated with hypoglycemia. Preterm newborns also may be at risk for developing carnitine deficiency because immature renal tubular function combined with impaired carnitine biosynthesis renders them strictly dependent on exogenous supplies to maintain normal plasma carnitine levels.

Valproic acid may cause an acquired type of secondary carnitine deficiency by directly impairing renal tubular reabsorption of carnitine. The effect on carnitine uptake and the existence of an underlying inborn error involving energy metabolism may be fatal; in other cases, it may primarily affect the muscle, causing weakness.

Frequency:

Mortality/Morbidity:

Race: Ethnic origins vary, and, in some families, consanguinity is present in cases of primary carnitine deficiency. Regarding fatty acid oxidation disorders as a cause of secondary carnitine deficiency, the most frequent cause of these disorders is MCAD deficiency, with an estimated frequency of 1 in 17,000 population of Northern European ancestry.

Sex: No sexual predilection exists for primary carnitine deficiency (an autosomal recessive disorder) or secondary carnitine deficiency.

Age:

History:

Physical: