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General description of the gene and the encoded protein(s) using information from HGNC and Ensembl, as well as predictions made by the Human Protein Atlas project.
Gene namei
Official gene symbol, which is typically a short form of the gene name, according to HGNC.
Assigned HPA protein class(es) for the encoded protein(s).
Disease related genes Enzymes Human disease related genes Metabolic proteins Potential drug targets
Predicted locationi
All transcripts of all genes have been analyzed regarding the location(s) of corresponding protein based on prediction methods for signal peptides and transmembrane regions.
Genes with at least one transcript predicted to encode a secreted protein, according to prediction methods or to UniProt location data, have been further annotated and classified with the aim to determine if the corresponding protein(s) are secreted or actually retained in intracellular locations or membrane-attached.
Remaining genes, with no transcript predicted to encode a secreted protein, will be assigned the prediction-based location(s).
The annotated location overrules the predicted location, so that a gene encoding a predicted secreted protein that has been annotated as intracellular will have intracellular as the final location.
Gene information from Ensembl and Entrez, as well as links to available gene identifiers are displayed here. Information was retrieved from Ensembl if not indicated otherwise.
Chromosome
17
Cytoband
p13.1
Chromosome location (bp)
7217125 - 7225266
Number of transcriptsi
Number of protein-coding transcripts from the gene as defined by Ensembl.
Useful information about the protein provided by UniProt.
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 1,2,3,4,5. 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 6,7,8,9. 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 10,11....show less
Molecular function (UniProt)i
Keywords assigned by UniProt to proteins due to their particular molecular function.
Oxidoreductase
Biological process (UniProt)i
Keywords assigned by UniProt to proteins because they are involved in a particular biological process.
Fatty acid metabolism, Lipid metabolism
Ligand (UniProt)i
Keywords assigned by UniProt to proteins because they bind, are associated with, or whose activity is dependent of some molecule.
FAD, Flavoprotein
Gene summary (Entrez)i
Useful information about the gene from Entrez
The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]...show less
PROTEIN INFORMATIONi
The protein information section displays alternative protein-coding transcripts (splice variants) encoded by this gene according to the Ensembl database.
The Splice variant identifier links to the Ensembl website protein summary for the selected splice variant. The data in the Swissprot and TrEMBL columns links to corresponding pages in the UniProt database.
The protein classes assigned to this protein are shown if expanding the data in the protein class column. Parent protein classes are in bold font and subclasses are listed under the parent class.
The length of the protein (amino acid residues according to Ensembl), molecular mass (kDalton), predicted signal peptide and number of predicted transmembrane region(s) according to in-house majority decision methods based on sets of predictors are also reported.
P49748 [Direct mapping] Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
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Enzymes ENZYME proteins Oxidoreductases Metabolic proteins MEMSAT-SVM predicted membrane proteins SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Disease related genes Potential drug targets Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0000062[fatty-acyl-CoA binding] GO:0001659[temperature homeostasis] GO:0003995[acyl-CoA dehydrogenase activity] GO:0004466[long-chain-acyl-CoA dehydrogenase activity] GO:0005515[protein binding] GO:0005654[nucleoplasm] GO:0005730[nucleolus] GO:0005739[mitochondrion] GO:0005743[mitochondrial inner membrane] GO:0005759[mitochondrial matrix] GO:0006629[lipid metabolic process] GO:0006631[fatty acid metabolic process] GO:0015980[energy derivation by oxidation of organic compounds] GO:0016020[membrane] GO:0016491[oxidoreductase activity] GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0017099[very-long-chain-acyl-CoA dehydrogenase activity] GO:0030855[epithelial cell differentiation] GO:0031314[extrinsic component of mitochondrial inner membrane] GO:0031966[mitochondrial membrane] GO:0033539[fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0042645[mitochondrial nucleoid] GO:0042802[identical protein binding] GO:0045717[negative regulation of fatty acid biosynthetic process] GO:0046322[negative regulation of fatty acid oxidation] GO:0050660[flavin adenine dinucleotide binding] GO:0090181[regulation of cholesterol metabolic process]
P49748 [Direct mapping] Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
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Enzymes ENZYME proteins Oxidoreductases Metabolic proteins MEMSAT-SVM predicted membrane proteins SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Disease related genes Potential drug targets Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0000062[fatty-acyl-CoA binding] GO:0001659[temperature homeostasis] GO:0003995[acyl-CoA dehydrogenase activity] GO:0004466[long-chain-acyl-CoA dehydrogenase activity] GO:0005515[protein binding] GO:0005654[nucleoplasm] GO:0005730[nucleolus] GO:0005739[mitochondrion] GO:0005743[mitochondrial inner membrane] GO:0005759[mitochondrial matrix] GO:0006629[lipid metabolic process] GO:0006631[fatty acid metabolic process] GO:0009062[fatty acid catabolic process] GO:0009409[response to cold] GO:0015980[energy derivation by oxidation of organic compounds] GO:0016020[membrane] GO:0016491[oxidoreductase activity] GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0017099[very-long-chain-acyl-CoA dehydrogenase activity] GO:0030855[epithelial cell differentiation] GO:0031314[extrinsic component of mitochondrial inner membrane] GO:0031966[mitochondrial membrane] GO:0033539[fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0042645[mitochondrial nucleoid] GO:0042802[identical protein binding] GO:0045717[negative regulation of fatty acid biosynthetic process] GO:0046322[negative regulation of fatty acid oxidation] GO:0050660[flavin adenine dinucleotide binding] GO:0090181[regulation of cholesterol metabolic process]
G3V1M7 [Direct mapping] Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial
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Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Protein evidence (Ezkurdia et al 2014)
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GO:0003995[acyl-CoA dehydrogenase activity] GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors]
P49748 [Direct mapping] Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
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Enzymes ENZYME proteins Oxidoreductases Metabolic proteins MEMSAT-SVM predicted membrane proteins SPOCTOPUS predicted membrane proteins Phobius predicted secreted proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Disease related genes Potential drug targets Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Mapped to neXtProt neXtProt - Evidence at protein level Protein evidence (Ezkurdia et al 2014)
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GO:0000062[fatty-acyl-CoA binding] GO:0001659[temperature homeostasis] GO:0003995[acyl-CoA dehydrogenase activity] GO:0004466[long-chain-acyl-CoA dehydrogenase activity] GO:0005515[protein binding] GO:0005654[nucleoplasm] GO:0005730[nucleolus] GO:0005739[mitochondrion] GO:0005743[mitochondrial inner membrane] GO:0005759[mitochondrial matrix] GO:0006629[lipid metabolic process] GO:0006631[fatty acid metabolic process] GO:0015980[energy derivation by oxidation of organic compounds] GO:0016020[membrane] GO:0016491[oxidoreductase activity] GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0017099[very-long-chain-acyl-CoA dehydrogenase activity] GO:0030855[epithelial cell differentiation] GO:0031314[extrinsic component of mitochondrial inner membrane] GO:0031966[mitochondrial membrane] GO:0033539[fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0042645[mitochondrial nucleoid] GO:0042802[identical protein binding] GO:0045717[negative regulation of fatty acid biosynthetic process] GO:0046322[negative regulation of fatty acid oxidation] GO:0050660[flavin adenine dinucleotide binding] GO:0090181[regulation of cholesterol metabolic process]
J3QRJ8 [Direct mapping] Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial
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Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Protein evidence (Ezkurdia et al 2014)
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GO:0003995[acyl-CoA dehydrogenase activity] GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors]
J3QKU9 [Direct mapping] Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial
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Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Protein evidence (Ezkurdia et al 2014)
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GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0050660[flavin adenine dinucleotide binding]
K7EQP4 [Direct mapping] Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial
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Metabolic proteins MEMSAT3 predicted membrane proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Protein evidence (Ezkurdia et al 2014)
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GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0050660[flavin adenine dinucleotide binding]
K7EJW8 [Direct mapping] Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial
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Metabolic proteins Predicted intracellular proteins Intracellular proteins predicted by MDM and MDSEC Human disease related genes Congenital disorders of metabolism Congenital disorders of lipid/glycolipid metabolism Protein evidence (Ezkurdia et al 2014)
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GO:0016627[oxidoreductase activity, acting on the CH-CH group of donors] GO:0050660[flavin adenine dinucleotide binding]
The Human Protein Atlas project is funded
