1.3.8.8: long-chain acyl-CoA dehydrogenase
This is an abbreviated version!
For detailed information about long-chain acyl-CoA dehydrogenase, go to the full flat file.
Word Map on EC 1.3.8.8
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1.3.8.8
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medium-chain
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beta-oxidation
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carnitine
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acylcarnitine
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cardiomyopathy
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cardiac
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peroxisome
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triglyceride
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rhabdomyolysis
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hypoglycemia
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acadvl
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palmitoyltransferase
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proliferator-activated
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inborn
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trifunctional
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nbs
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3-hydroxyacyl-coa
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acidurias
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myopathic
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hypoketotic
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glutaric
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myoglobinuria
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dehydrogenase-deficient
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isovaleryl-coa
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bezafibrate
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lchad
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faods
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medicine
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mcadd
- 1.3.8.8
-
medium-chain
-
beta-oxidation
- carnitine
- acylcarnitine
- cardiomyopathy
- cardiac
- peroxisome
- triglyceride
- rhabdomyolysis
- hypoglycemia
- acadvl
- palmitoyltransferase
-
proliferator-activated
-
inborn
-
trifunctional
- nbs
- 3-hydroxyacyl-coa
- acidurias
-
myopathic
-
hypoketotic
-
glutaric
- myoglobinuria
-
dehydrogenase-deficient
- isovaleryl-coa
- bezafibrate
- lchad
-
faods
- medicine
-
mcadd
Reaction
Synonyms
ACAD 9, AcdB, acyl-CoA dehydrogenase 9, BACH, CTE-II, EC 1.3.99.13, EC 1.3.99.3, LCAD, long chain acyl coenzyme A dehydrogenase, long chain acyl-CoA dehydrogenase, long-chain acyl-CoA dehydrogenase, long-chain acyl-CoA hydrolase, long-chain acyl-coenzyme A dehydrogenase, MCAD, medium long-chain acyl-CoA dehydrogenase, More, MTE-II, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, PtmO4, type-II acyl-CoA thioesterase, very long chain acyl-CoA dehydrogenase, very long-chain acyl-CoA dehydrogenase, very-long-chain acyl-CoA dehydrogenase, very-long-chain acyl-coenzyme A dehydrogenase, VLCAD, VLMCAD
ECTree
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Natural Substrates Products
Natural Substrates Products on EC 1.3.8.8 - long-chain acyl-CoA dehydrogenase
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REACTION DIAGRAM
2,6-dimethylheptanoyl-CoA + oxidized flavin
2,3-dehydro-2,6-dimethylheptanoyl-CoA + reduced flavin
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specific LCAD substrate
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palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
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enzyme does not affect all acyl-CoA utilizing cellular processes
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2-dodecenoyl-CoA + reduced acceptor
enzyme shows preference for long-chain fatty acids
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lauroyl-CoA + acceptor
2-dodecenoyl-CoA + reduced acceptor
enzyme shows preference for long-chain fatty acids
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?
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enzyme deficiency may ba a cause of autism, physiological alterations in case of enzyme deficiency are in TCA energy metabolism, ammonia detoxification, and synthesis of omega-3 DHA, and elevated acyl-carnitine levels, the mitochondrial enzyme is responsible for beta-oxidation of branched and unsaturated fatty acids with preference for long-chain fatty acids
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additional information
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catalyses the initial step of mitochondrial beta-oxidation of long-chain fatty acids with a chain length of 14 to 20 carbons
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