Function and Biology

Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin

Source organism: Homo sapiens
Biochemical function: hydrolase activity
Biological process: branched-chain amino acid catabolic process
Cellular component: mitochondrial matrix

EC 3.1.2.4: 3-hydroxyisobutyryl-CoA hydrolase

Reaction catalysed:
3-hydroxy-2-methylpropanoyl-CoA + H(2)O = CoA + 3-hydroxy-2-methylpropanoate
Systematic name:
3-hydroxy-2-methylpropanoyl-CoA hydrolase
Alternative Name(s):
  • 3-hydroxy-isobutyryl CoA hydrolase
  • HIB CoA deacylase

Sequence family

Pfam Protein family (Pfam)
PF16113
Domain description: Enoyl-CoA hydratase/isomerase
Occurring in:
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
The deposited structure of PDB entry 3bpt contains 1 copy of Pfam domain PF16113 (Enoyl-CoA hydratase/isomerase) in 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR045004
Domain description: Enoyl-CoA hydratase/isomerase domain
Occurring in:
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
IPR029045
Domain description: ClpP/crotonase-like domain superfamily
Occurring in:
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
IPR032259
Domain description: Enoyl-CoA hydratase/isomerase, HIBYL-CoA-H type
Occurring in:
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

Structure domain

CATH CATH domain
3.90.226.10
Class: Alpha Beta
Architecture: Alpha-Beta Complex
Topology: 2-enoyl-CoA Hydratase; Chain A, domain 1
Homology: 2-enoyl-CoA Hydratase; Chain A, domain 1
Occurring in:
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
The deposited structure of PDB entry 3bpt contains 1 copy of CATH domain 3.90.226.10 (2-enoyl-CoA Hydratase; Chain A, domain 1) in 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial. Showing 1 copy in chain A.