InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Gene: HADHB

Summary

InnateDB Gene

IDBG-35294.6

Last Modified

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

Gene Symbol

HADHB

Gene Name

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

Synonyms

ECHB; MTPB; TP-BETA

Species

Homo sapiens

Ensembl Gene

ENSG00000138029

Encoded Proteins

IDBP-35298

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

IDBP-294709

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

IDBP-367729

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

IDBP-367804

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

IDBP-367807

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

IDBP-596838

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

IDBP-595168

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

Protein Structure

Useful resources

Stemformatics EHFPI ImmGen

Entrez Gene

Summary

This gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. [provided by RefSeq, Jul 2008]
This gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013]

Gene Information

Type

Protein coding

Genomic Location

Chromosome 2:26243170-26290468

Strand

Forward strand

Band

p23.3

Transcripts

ENST00000317799	ENSP00000325136
ENST00000405867	ENSP00000385411
ENST00000448743	ENSP00000415300
ENST00000425035	ENSP00000404633
ENST00000412805	ENSP00000413103
ENST00000479347
ENST00000494615
ENST00000537713	ENSP00000444295
ENST00000545822	ENSP00000442665

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 1 interaction(s) predicted by orthology.

Experimentally validated
Total	59 [view]
Protein-Protein	56 [view]
Protein-DNA	3 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [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:0003988	acetyl-CoA C-acyltransferase activity
GO:0004300	enoyl-CoA hydratase activity
GO:0005515	protein binding
GO:0016508	long-chain-enoyl-CoA hydratase activity
GO:0016509	long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0016747	transferase activity, transferring acyl groups other than amino-acyl groups
GO:0032403	protein complex binding
GO:0044822	poly(A) RNA binding
GO:0051287	NAD binding

Biological Process

GO:0006635	fatty acid beta-oxidation
GO:0006644	phospholipid metabolic process
GO:0008152	metabolic process
GO:0035965	cardiolipin acyl-chain remodeling
GO:0044255	cellular lipid metabolic process
GO:0044281	small molecule metabolic process
GO:0046474	glycerophospholipid biosynthetic process

Cellular Component

GO:0005739	mitochondrion
GO:0005740	mitochondrial envelope
GO:0005741	mitochondrial outer membrane
GO:0005743	mitochondrial inner membrane
GO:0005783	endoplasmic reticulum
GO:0016507	mitochondrial fatty acid beta-oxidation multienzyme complex
GO:0042645	mitochondrial nucleoid
GO:0070062	extracellular vesicular exosome