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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

Reaction

a long-chain acyl-CoA
+
electron-transfer flavoprotein
=
a long-chain trans-2,3-dehydroacyl-CoA
+
reduced electron-transfer flavoprotein

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

     1 Oxidoreductases
         1.3 Acting on the CH-CH group of donors
             1.3.8 With a flavin as acceptor
                1.3.8.8 long-chain acyl-CoA dehydrogenase

Engineering

Engineering on EC 1.3.8.8 - long-chain acyl-CoA dehydrogenase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
997insT
-
frameshift, heterozygote mutation of exon 10, mutation of a myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency
A364G
-
patient died suddenly in infancy, severe VLCAD deficiency
A450P
A985G
-
nearly all patients with residual MCAD activities below 2.5% are homozygous 985A>G carriers
E261Q
-
mutant exhibits less than 0.02% of the wild-type activity
G520A
-
patient symptomatic, with episodic rhabdomyolysis
K259M
missense mutation leading to VLCAD deficiency
K264E
-
A790G, heterozygote mutation of exon 9
L462P
L462Q
missense mutation leading to VLCAD deficiency
R429W
R573W
T199C
-
all patients with the 199T>C mutation have residual activities above 10%
T220M
T848C
-
patients asymptomatic on dietary management
V243A
additional information