Gene: [02q3/ACADL] acyl-CoA dehydrogenase L (fatty acid long straight-chain C8 to C16); hypoglycemia, intermittent non-ketotic (LCAD deficiency); dicarboxylicaciduria (LCAD deficiency);

FUN

[1] Acyl-CoA dehydrogenase L participates in the beta-oxidation of fatty acids with long straight-chain (C8-C16). The reaction occurs in the complex with ETF - the electron transfer flavoprotein (GEM:15q2/ETFA).
[2] The reaction catalyzed: acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.
[3] Cofactor: FAD."

MOP

Acyl-CoA dehydrogenase L is a flavoprotein.

FAG

On other enzymes from the acyl-CoA dehydrogenase group, see in GEM:01p31/ACADM.

HET

On the various genetic disorders of mitochondrial beta-oxidation of fatty acids, see GEM:01p31/ACADM and GEM:0X^/ACADX."

REF

PHE,PAT,MEB,FOG "Hale &: Pediatr Res, 19, 666-671, 1985
PHE,PAT,MEB,FOG "Naylor &: J Inherit Metab Dis, 3, 19-24, 1980
REV,PEP,MEB,PAT "Tanaka K &: Enzyme, 38, 91-107, 1987

SWI

 SWISSPROT: P28330

KEY

neu, mtbd, mito

CLA

coding, basic

LOC

02 q34-35

MIM

 MIM: 201460

EZN

 ENZYME: 1.3.99.13