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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Specific Activity
Specific Activity on EC 1.3.8.8 - long-chain acyl-CoA dehydrogenase
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0.00034
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crude extract, strain TH183, genotype fadR251(S), acetate and oleate as carbon source
0.00052
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crude extract, strain TH18, genotype fadR251(S), acetate as carbon source
0.00064
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crude extract, strain TH182, genotype fadR250(S) acetate as carbon source
0.00083
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crude extract, strain TH182, genotype fadR250(S), acetate and oleate as carbon source
0.0011
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crude extract, strain TH181, genotype fadR+, acetate as carbon source
0.0038
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crude extract, strain TH181, genotype fadR+, acetate and oleate as carbon source
0.0046
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crude extract, strain RS3010, genotype fadR1, acetate as carbon source
0.0055
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crude extract, strain RS3010, genotype fadR1, acetate and oleate as carbon source
0.06037
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LCAD enzyme activity in livers of partially deficient LCAD mice 8 weeks after injection of recombinant adeno-associated viral mLCAD vector via the hepatic portal vein
0.1
0.2546
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LCAD enzyme activity in tibialis anterior muscle of LCAD-heterozygous deficient mice 10 weeks post-injection 1 x 10exp11 vg recombinant adeno-associated viral mLCAD
1.5
additional information
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heterozygous ACADVL mutation carriers show residual VLCAD activities of 23.7 to 54.2%
additional information
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residual VLCAD activity of patients with confirmed VLCAD-deficiency is between 0.3 and 1.1%