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Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
External IDs OMIM143450 MGI3651143 HomoloGene153 GeneCards: HADHB Gene
EC number
Species Human Mouse
Entrez 3032 231086
Ensembl ENSG00000138029 ENSMUSG00000059447
UniProt P55084 Q99JY0
RefSeq (mRNA) NM_000183 NM_145558
RefSeq (protein) NP_000174 NP_663533
Location (UCSC) Chr 2:
26.47 – 26.51 Mb
Chr 5:
30.16 – 30.18 Mb
PubMed search [1] [2]

Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase, acetyl-CoA acyltransferase, or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene.[1]

HADHB is a subunit of the mitochondrial trifunctional protein and has thiolase activity.


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.[1]


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This article incorporates text from the United States National Library of Medicine, which is in the public domain.