InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Gene: HADHA

Summary

InnateDB Gene

IDBG-35125.6

Last Modified

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

Gene Symbol

HADHA

Gene Name

hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit

Synonyms

ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA

Species

Homo sapiens

Ensembl Gene

ENSG00000084754

Encoded Proteins

IDBP-35127

hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit

IDBP-583762

hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit

Protein Structure

Useful resources

Stemformatics EHFPI ImmGen

Entrez Gene

Summary

This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]

Gene Information

Type

Protein coding

Genomic Location

Chromosome 2:26190635-26244726

Strand

Reverse strand

Band

p23.3

Transcripts

ENST00000380649	ENSP00000370023
ENST00000492433	ENSP00000438039
ENST00000471743
ENST00000461025

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 59 experimentally validated interaction(s) in this database.
They are also associated with 3 interaction(s) predicted by orthology.

Experimentally validated
Total	59 [view]
Protein-Protein	57 [view]
Protein-DNA	2 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	3 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000062	fatty-acyl-CoA binding
GO:0003824	catalytic activity
GO:0003857	3-hydroxyacyl-CoA dehydrogenase activity
GO:0003985	acetyl-CoA C-acetyltransferase activity
GO:0003988	acetyl-CoA C-acyltransferase activity
GO:0004300	enoyl-CoA hydratase activity
GO:0005515	protein binding
GO:0016491	oxidoreductase activity
GO:0016508	long-chain-enoyl-CoA hydratase activity
GO:0016509	long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0032403	protein complex binding
GO:0050662	coenzyme binding
GO:0051287	NAD binding

Biological Process

GO:0006631	fatty acid metabolic process
GO:0006635	fatty acid beta-oxidation
GO:0006644	phospholipid metabolic process
GO:0008152	metabolic process
GO:0032868	response to insulin
GO:0035965	cardiolipin acyl-chain remodeling
GO:0042493	response to drug
GO:0044255	cellular lipid metabolic process
GO:0044281	small molecule metabolic process
GO:0046474	glycerophospholipid biosynthetic process
GO:0055114	oxidation-reduction process

Cellular Component

GO:0005739	mitochondrion
GO:0005743	mitochondrial inner membrane
GO:0016507	mitochondrial fatty acid beta-oxidation multienzyme complex
GO:0042645	mitochondrial nucleoid