InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: ACADSB

Summary

InnateDB Protein

IDBP-92409.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

ACADSB

Protein Name

acyl-CoA dehydrogenase, short/branched chain

Synonyms

2-MEBCAD; ACAD7; SBCAD;

Species

Homo sapiens

Ensembl Protein

ENSP00000357873

InnateDB Gene

IDBG-92407 (ACADSB)

Protein Structure

UniProt Annotation

Function

Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.

Subcellular Localization

Mitochondrion matrix.

Disease Associations

Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD) [MIM:610006]: Autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2- methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. {ECO:0000269PubMed:10832746, ECO:0000269PubMed:16317551}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Ubiquitous.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 5 experimentally validated interaction(s) in this database.

Experimentally validated
Total	5 [view]
Protein-Protein	4 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003995	acyl-CoA dehydrogenase activity
GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors
GO:0050660	flavin adenine dinucleotide binding

Biological Process

GO:0006631	fatty acid metabolic process
GO:0008152	metabolic process
GO:0009083	branched-chain amino acid catabolic process
GO:0034641	cellular nitrogen compound metabolic process
GO:0044281	small molecule metabolic process
GO:0055114	oxidation-reduction process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR013107 Acyl-CoA dehydrogenase, C-terminal domain
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal

PFAM

PF02770
PF00441
PF08028
PF02771

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P45954

PhosphoSite