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  • carnitine palmitoyl transferase, deficiency
    Carnitine palmitoyl transferase, deficiency
  • plasma membrane carnitine transport defect
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  • dehydratase
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  • threonine dehydratase
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ALCAR acetyl-L-carnitine
CPT carnitine palmityl transferase; carotid pulse tracing; chest physiotherapy; child protection team; c...
MCD magnetic circular dichroism; mast-cell degranulation; mean cell diameter; mean of consecutive differ...
SCD scleroderma; service-connected disability; sickle-cell disease; spinocerebellar degeneration; subacu...
ALAD ALA Dehydratase
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ALAD 5-Aminolaevulinate dehydratase
ALAD 5-Aminolaevulinic acid dehydratase
ALAD Delta-aminolevulinate dehydratase
ALA.D delta-amino-laevulinic acid dehydratase
Delta-ALAD Delta-aminolevulinic acid dehydratase
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    ºñŸÀÎ À¯µµÃ¼. ¸», µÅÁö, ¼ÒÀÇ °ñ°Ý±Ù¿¡ Á¸ÀçÇÑ´Ù. À̰ÍÀ» µ¿¹°¿¡ ÁÖ»çÇÏ¸é ±¸Åä, ¼³»ç, À¯¿¬, »êµ¿ µîÀ» ÀÏÀ¸Å²´Ù. Ç×°©»ó¼±Á¦·Î »ç¿ëµÈ´Ù.
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carnitine dehydratase <enzyme> From E coli; catalyses the reversible dehydration of (l)-carnitine forming crotonobetaine
Registry number: EC 4.2.1.-
Synonym: caib protein, caib gene product
(26 Jun 1999)
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carnitine <biochemistry> _ Hydroxy _ trimethyl aminobutyric acid. Compound that transports long chain fatty acids across the inner mitochondrial membrane in the form of acyl carnitine. Sometimes referred to as Vitamin Bt or Vitamin B7.
(18 Nov 1997)
carnitine acyltransferases <enzyme> Acyltransferases in the inner mitochondrial membrane that catalyze the reversible transfer of acyl groups from acyl-CoA to l-carnitine and thereby mediate the transport of activated fatty acids through that membrane.
Registry number: EC 2.3.1.-
(12 Dec 1998)
carnitine amidase <enzyme> Hydrolyzes l-carnitine amide steroselectively
Registry number: EC 3.5.1.-
(26 Jun 1999)
carnitine coenzyme A ligase <enzyme> Caic protein isolated from e. Coli
Registry number: EC 6.2.1.-
Synonym: carnitine CoA ligase, crotonobetaine CoA ligase, caic gene product
(26 Jun 1999)
carnitine o-acetyltransferase <enzyme> An enzyme that catalyses the formation of o-acetylcarnitine from acetyl-CoA plus carnitine.
Chemical name: Acetyl-CoA:carnitine O-acetyltransferase
Registry number: EC 2.3.1.7
(12 Dec 1998)
carnitine o-palmitoyltransferase <enzyme> An enzyme that catalyses reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane.
Chemical name: Palmitoyl-CoA:L-carnitine O-palmitoyltransferase
Registry number: EC 2.3.1.21
(12 Dec 1998)
carnitine palmitoyltransferase <enzyme> An enzyme that reversibly forms acylcarnitines and coenzyme A from carnitine and acylcoenzyme A (often, palmitoyl-CoA); important in fatty acid oxidation. Deficiency of isozyme I results in ketogenesis with hypoglycaemia; deficiency of isozyme II affects primarily skeletal muscle.
(05 Mar 2000)
carnitine racemase <enzyme> Homologous to enoyl hydratases and isomerases; converts d(+)-carnitine to l(-)-carnitine; isolated from e. Coli
Registry number: EC 5.1.-
Synonym: caid gene product
(26 Jun 1999)
l-carnitine <amino acid> Is a dipeptide - an amino acid made from two other aminos, methionine and lysine. It can be synthesised in the liver if sufficient amounts of lysine, B1, B6 and iron are available. Muscle and organ meat, fish and milk products are the best sources of carnitine in the diet.
Carnitine has been shown to have a major role in the metabolism of fat and in the reduction of triglycerides by increasing fat utilisation. It transfers fatty acids across the membranes of the mitochondria where they can be utilised as sources of energy. It also increases the rate at which the liver uses fats. By preventing fatty build-up, this amino acid aids in weight loss and decreases the risk of heart disease. Carnitine has been shown to be deficient in hearts of patients who have died of acute myocardial infections.
Muscular dystrophy and myotonic dystrophy have been shown to lead to carnitine loss in the urine and therefore higher requirements for it. Carnitine is stored primarily in the skeletal muscles and heart, where it is needed to transform fatty acids into energy for muscular activity. It is also concentrated in sperm and the brain. Many athletes have noted increased endurance and muscle building with carnitine supplementation.
Deficiencies may increase symptoms of fatigue, angina, muscle weakness or confusion. A low level of Vitamin C will also result in apparent Carnitine deficiency. It is contraindicated for people with liver or kidney disease or diabetes It has proven helpful in improving lipid metabolism and reducing elevated total lipids, cholesterol and triglycerides in people with cardiac problems and diabetes, but should only be taken with medical supervision in these conditions.
(25 Jun 1999)
bile acid 7alpha-dehydratase <enzyme> Enzyme in the bacterial bile acid 7alpha-dehydroxylation pathway
Registry number: EC 4.2.1.-
Synonym: c24 bile acid 7alpha-dehydratase, ba7alphad, baie gene product
(26 Jun 1999)
carbonate dehydratase <enzyme> A zinc-containing enzyme of erythrocytes with molecular weight of 30 kD. It is among the most active of known enzymes and catalyses the reversible hydration of carbon dioxide, which is significant in the transport of co2 from the tissues to the lungs. The enzyme is inhibited by acetazolamide.
Chemical name: Carbonate hydro-lyase
Registry number: EC 4.2.1.1
(12 Dec 1998)
carbonate dehydratase inhibitor An agent, usually chemically related to the sulfonamides, that inhibits the activity of carbonate dehydratase, producing a general decrease in the formation of H2CO3 in the tissues.
See: acetazolamide, dichlorphenamide.
Synonym: carbonic anhydrase inhibitor.
(05 Mar 2000)
galactonate dehydratase <enzyme> In mutant of klebsiella pneumoniae also functions in catabolism of d-fuconate
Registry number: EC 4.2.1.6
Synonym: galactonate dehydrase, d-galactonate dehydratase, d-galactonate-d-fuconate dehydratase
(26 Jun 1999)
glycerol dehydratase <enzyme> Requires a cobamide coenzyme
Registry number: EC 4.2.1.30
(26 Jun 1999)
retinol dehydratase <enzyme> Catalyses conversion of retinol to anhydroretinol; isolated from spodoptera frugiperda; genbank u28654
Registry number: EC 4.2.1.-
(26 Jun 1999)
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