Protein: Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9) (P49748)
Organism: Homo sapiens (Human)
Function: Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains.
Subcellular location: Mitochondrion inner membrane {ECO:0000269|PubMed:9461620, ECO:0000269|PubMed:9599005}; Peripheral membrane protein {ECO:0000269|PubMed:9599005}.; SUBCELLULAR LOCATION: [Isoform 2]: Mitochondrion inner membrane {ECO:0000269|PubMed:17374501}; Peripheral membrane protein {ECO:0000269|PubMed:17374501}.
Sequence: MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPYAGGAAQLALDKSDSHPSDALTRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPAKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
InterPro domains: - IPR037069: Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamily (homologous_superfamily) [91-211]
- IPR013786: Acyl-CoA dehydrogenase/oxidase, N-terminal (domain) [103-209]
- IPR036250: Acyl-CoA dehydrogenase-like, C-terminal (homologous_superfamily) [325-473]
- IPR006091: Acyl-CoA oxidase/dehydrogenase, middle domain (domain) [213-315]
- IPR049448: ACAD9/ACADV-like, C-terminal domain (domain) [527-647]
- IPR009100: Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamily (homologous_superfamily) [88-340]
- IPR006089: Acyl-CoA dehydrogenase, conserved site (conserved_site) [215-454]
- IPR046373: Acyl-CoA oxidase/dehydrogenase, middle domain superfamily (homologous_superfamily) [212-326]
- IPR009075: Acyl-CoA dehydrogenase/oxidase, C-terminal (domain) [327-473]
Interaction partners: - Trifunctional enzyme subunit alpha, mitochondrial
- Carnitine O-palmitoyltransferase 2, mitochondrial
- Trifunctional enzyme subunit beta, mitochondrial
- 3-ketoacyl-CoA thiolase, mitochondrial
- Enoyl-CoA hydratase, mitochondrial
- 3-ketoacyl-CoA thiolase, peroxisomal
- Peroxisomal bifunctional enzyme
- Peroxisomal acyl-coenzyme A oxidase 1
- Long-chain specific acyl-CoA dehydrogenase, mitochondrial
- Peroxisomal acyl-coenzyme A oxidase 3
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016787 (hydrolase activity), GO:0005515 (protein binding), GO:0016788 (hydrolase activity, acting on ester bonds), GO:0016627 (oxidoreductase activity, acting on the CH-CH group of donors), GO:0052890 (oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor), GO:0003995 (acyl-CoA dehydrogenase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:0044255 (cellular lipid metabolic process), GO:0006629 (lipid metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0044248 (cellular catabolic process), GO:0006082 (organic acid metabolic process), GO:0030258 (lipid modification), GO:0006631 (fatty acid metabolic process), GO:0016054 (organic acid catabolic process), GO:0044242 (cellular lipid catabolic process), GO:0043436 (oxoacid metabolic process), GO:0016042 (lipid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0046395 (carboxylic acid catabolic process), GO:0034440 (lipid oxidation), GO:0019395 (fatty acid oxidation), GO:0009062 (fatty acid catabolic process), GO:0072329 (monocarboxylic acid catabolic process), GO:0006635 (fatty acid beta-oxidation), GO:0032787 (monocarboxylic acid metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0031975 (envelope), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0005739 (mitochondrion), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0019866 (organelle inner membrane), GO:0043231 (intracellular membrane-bounded organelle), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane)