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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 **6 - 10** of 16 in total

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GO Term	Evidence Code	PMID
epithelial cell differentiation	IEP
fatty acid beta-oxidation using acyl-CoA dehydrogenase	ISS
fatty acid beta-oxidation using acyl-CoA dehydrogenase	IDA
fatty acid beta-oxidation using acyl-CoA dehydrogenase	IMP
fatty acid beta-oxidation using acyl-CoA dehydrogenase	IEA

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 **1 - 5** of 10 in total

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GO Term	Evidence Code	PMID
nucleoplasm
nucleolus
mitochondrion	ISS
mitochondrion	IDA
mitochondrion	HTP

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.

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