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The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0000062 [fatty-acyl-CoA binding] GO:0003995 [acyl-CoA dehydrogenase activity] GO:0004361 [glutaryl-CoA dehydrogenase activity] GO:0005739 [mitochondrion] GO:0005743 [mitochondrial inner membrane] GO:0005759 [mitochondrial matrix] GO:0006554 [lysine catabolic process] GO:0006568 [tryptophan metabolic process] GO:0006637 [acyl-CoA metabolic process] GO:0008152 [metabolic process] GO:0009055 [electron carrier activity] GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors] GO:0019395 [fatty acid oxidation] GO:0033539 [fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0046949 [fatty-acyl-CoA biosynthetic process] GO:0050660 [flavin adenine dinucleotide binding] GO:0052890 [oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor] GO:0055088 [lipid homeostasis] GO:0055114 [oxidation-reduction process]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Disease related genes Potential drug targets Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000062 [fatty-acyl-CoA binding] GO:0003995 [acyl-CoA dehydrogenase activity] GO:0004361 [glutaryl-CoA dehydrogenase activity] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006554 [lysine catabolic process] GO:0006568 [tryptophan metabolic process] GO:0008152 [metabolic process] GO:0009055 [electron carrier activity] GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors] GO:0033539 [fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0050660 [flavin adenine dinucleotide binding] GO:0052890 [oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor] GO:0055088 [lipid homeostasis] GO:0055114 [oxidation-reduction process]
Predicted intracellular proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003995 [acyl-CoA dehydrogenase activity] GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors] GO:0055114 [oxidation-reduction process]
Predicted intracellular proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003995 [acyl-CoA dehydrogenase activity] GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors] GO:0055114 [oxidation-reduction process]
