Gene Information
Gene NameHIBCH
OrganismHomo sapiens (Human)
Gene Length386
Protein Names3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (EC 3.1.2.4) (3-hydroxyisobutyryl-coenzyme A hydrolase) (HIB-CoA hydrolase) (HIBYL-CoA-H)
Target Name3-hydroxyisobutyryl-coa hydrolase, mitochondrial
Target TypeNA
Gene Age1714 ( ENSG00000198130 )
Evolutionary StageEuk+Bac
AlphaFoldDBQ6NVY1
Gene CardHIBCH
Uniprot IDQ6NVY1
PfamPF16113
In ASDno
Allosteric Predictionno (-0.39094318)
Ortholog in AnimalsAlpaca_ HIBCH ( ortholog_one2one )
Dog_ HIBCH ( ortholog_one2one )
GuineaPig_ HIBCH ( ortholog_one2one )
Macaque_ HIBCH ( ortholog_one2one )
Mouse_ Hibch ( ortholog_one2one )
Pig_ HIBCH ( ortholog_one2one )
Rat_ Hibch ( ortholog_one2one )
Rabbit_ HIBCH ( ortholog_one2one )
OhnologsNA
ParalogsECI1, ECHS1, AUH, ECH1, CDY1, CDYL, CDY2A, CDY2B, CDY1B, CDYL2, ECHDC3, ECHDC2, ECHDC1
TissueNA
Tissue SpecificityHighly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. {ECO:0000269|PubMed:8824301}.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 3BPT  (X-ray  1.50A 
DBSNP rs1058180  rs121918329 
Subcellular location [CC]Mitochondrion {ECO:0000250}.
Mouse PG classificationNA
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