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acyl-CoA dehydrogenase very long chain

Summary

Gene Symbol

ACADVL

Aliases

ACAD6
LCACD
VLCAD

Organism

Homo sapiens (human)

External Links

NCBI Gene

HGNC

KEGG Gene ID

hsa:37

PubChem

Alliance of Genome Resources

HGNC:92

JoGo

ACADVL

TogoVar

ACADVL

Annotation

Keyword

3D-structure
Acetylation
Alternative splicing
Cardiomyopathy
Direct protein sequencing
Disease variant
FAD
Fatty acid metabolism
Mitochondrion inner membrane
Oxidoreductase
Phosphoprotein
Proteomics identification
Reference proteome
S-nitrosylation
Transit peptide

Proteins

Displaying **all 2** entries
UniProt	Protein Name
P49748	Long-chain acyl-CoA dehydrogenase, mitochondrial
B3KPA6

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 16 in total

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GO Term	Evidence Code	PMID
temperature homeostasis	ISS
temperature homeostasis	IEA
fatty acid beta-oxidation	IEA
response to cold
energy derivation by oxidation of organic compounds	TAS

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

generation of precursor metabolites and energy [inference]

energy derivation by oxidation of organic compounds

catabolic process [inference]

lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

primary metabolic process [inference]

lipid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

fatty acid oxidation [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

lipid modification [inference]

lipid oxidation [inference]

fatty acid oxidation [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

fatty acid oxidation [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation

fatty acid beta-oxidation using acyl-CoA dehydrogenase

cellular developmental process [inference]

cell differentiation [inference]

epithelial cell differentiation

multicellular organismal process [inference]

multicellular organismal-level homeostasis [inference]

temperature homeostasis

developmental process [inference]

cellular developmental process [inference]

cell differentiation [inference]

epithelial cell differentiation

homeostatic process [inference]

multicellular organismal-level homeostasis [inference]

temperature homeostasis

response to stimulus [inference]

response to stress [inference]

response to cold

response to abiotic stimulus [inference]

response to temperature stimulus [inference]

response to cold

biological regulation [inference]

regulation of biological process [inference]

negative regulation of biological process [inference]

negative regulation of cellular process [inference]

negative regulation of metabolic process [inference]

negative regulation of biosynthetic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of lipid metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of small molecule metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

regulation of cellular process [inference]

regulation of metabolic process [inference]

regulation of biosynthetic process [inference]

negative regulation of biosynthetic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

regulation of lipid biosynthetic process [inference]

regulation of fatty acid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of metabolic process [inference]

negative regulation of biosynthetic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of lipid metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of small molecule metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

regulation of ketone metabolic process [inference]

regulation of fatty acid metabolic process [inference]

regulation of fatty acid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of fatty acid metabolic process [inference]

regulation of fatty acid oxidation [inference]

negative regulation of fatty acid oxidation

regulation of small molecule metabolic process [inference]

regulation of fatty acid metabolic process [inference]

regulation of fatty acid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of fatty acid metabolic process [inference]

regulation of fatty acid oxidation [inference]

negative regulation of fatty acid oxidation

negative regulation of small molecule metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

regulation of cholesterol metabolic process

regulation of primary metabolic process [inference]

regulation of lipid metabolic process [inference]

regulation of fatty acid metabolic process [inference]

regulation of fatty acid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of fatty acid metabolic process [inference]

regulation of fatty acid oxidation [inference]

negative regulation of fatty acid oxidation

regulation of steroid metabolic process [inference]

regulation of cholesterol metabolic process

negative regulation of lipid metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

regulation of lipid biosynthetic process [inference]

regulation of fatty acid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of cellular process [inference]

negative regulation of metabolic process [inference]

negative regulation of biosynthetic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of lipid metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

negative regulation of lipid biosynthetic process [inference]

negative regulation of fatty acid biosynthetic process

negative regulation of small molecule metabolic process [inference]

negative regulation of fatty acid metabolic process [inference]

Displaying entries **6 - 10** of 10 in total

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GO Term	Evidence Code	PMID
mitochondrial inner membrane	IEA
mitochondrial matrix	TAS
mitochondrial membrane	IDA
mitochondrial membrane	IEA
mitochondrial nucleoid	IDA

GO Hierarchy

cellular component

cellular anatomical structure [inference]

nucleoplasm

nucleoid [inference]

mitochondrial nucleoid

membrane [inference]

organelle membrane [inference]

organelle inner membrane [inference]

mitochondrial inner membrane

mitochondrial membrane

mitochondrial inner membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

membraneless organelle [inference]

intracellular membraneless organelle [inference]

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular membraneless organelle [inference]

Displaying entries **1 - 5** of 14 in total

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GO Term	Evidence Code	PMID
fatty-acyl-CoA binding	IBA
acyl-CoA dehydrogenase activity	IMP
acyl-CoA dehydrogenase activity	IEA
long-chain fatty acyl-CoA dehydrogenase activity	TAS
long-chain fatty acyl-CoA dehydrogenase activity	IDA

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

oxidoreductase activity, acting on the CH-CH group of donors

oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor [inference]

acyl-CoA dehydrogenase activity

binding [inference]

protein binding

identical protein binding

lipid binding [inference]

fatty acid derivative binding [inference]

fatty-acyl-CoA binding

small molecule binding [inference]

ion binding [inference]

anion binding [inference]

flavin adenine dinucleotide binding

acyl-CoA binding [inference]

fatty-acyl-CoA binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

acyl-CoA binding [inference]

fatty-acyl-CoA binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

acyl-CoA binding [inference]

fatty-acyl-CoA binding

sulfur compound binding [inference]

acyl-CoA binding [inference]

fatty-acyl-CoA binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Acyl-CoA dehydrogenase

Functional Category

I: Lipid transport and metabolism
P: Inorganic ion transport and metabolism
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying **all 3** entries
Gene Ontology
flavin adenine dinucleotide binding
oxidoreductase activity, acting on the CH-CH group of donors
oxidoreductase activity

Displaying **all 9** entries
InterPro
ACAD9/ACADV-like, C-terminal domain
Acyl-CoA dehydrogenase, conserved site
Acyl-CoA dehydrogenase-like, C-terminal
Acyl-CoA dehydrogenase/oxidase, C-terminal
Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamily
Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamily
Acyl-CoA dehydrogenase/oxidase, N-terminal
Acyl-CoA oxidase/dehydrogenase, middle domain superfamily
Acyl-CoA oxidase/dehydrogenase, middle domain

Human Protein Atlas

ENSG00000072778

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K09479

Name

very long chain acyl-CoA dehydrogenase [EC:1.3.8.9]

References

Rhea

RHEA:19181

H⁺ + a very-long-chain 2,3-saturated fatty acyl-CoA

a very-long-chain (2E)-enoyl-CoA

Enzyme

1.3.8.9

PMID

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0080155	very long chain acyl-CoA dehydrogenase deficiency	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **1 - 10** of 62 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000252	Microcephaly
HP:0000256	Macrocephaly
HP:0000952	Jaundice
HP:0001252	Hypotonia
HP:0001254	Lethargy
HP:0001324	Muscle weakness
HP:0001397	Hepatic steatosis
HP:0001404	Hepatocellular necrosis
HP:0001405	Periportal fibrosis