Proteins/UNIPROT:Q6NVY1

HIBCH

ProteinUNIPROT:Q6NVY1

Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA..

Function from UniProtKB (Q6NVY1), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

3-hydroxyisobutyryl-CoA hydrolase deficiency has a germline basis in HIBCHDiseaseGenCC HGNC:4908|MONDO:0009603Inferred only
Leigh syndrome has a germline basis in HIBCHDiseaseGenCC HGNC:4908|MONDO:0009723Inferred only

Statements

25 statements

25 statements · 4 reference databases · Subject: HIBCH · Sources: Hetionet, Reactome, Rhea, WikiPathways

Order

BPIFB1 covaries with HIBCHProteinHetionet hetionet:covaries:26275-92747Inferred only
OR14C36 covaries with HIBCHProteinHetionet hetionet:covaries:127066-26275Inferred only
GRB2 interacts with HIBCHProteinHetionet hetionet:interacts:26275-2885Inferred only
UBC interacts with HIBCHProteinHetionet hetionet:interacts:26275-7316Inferred only
HIBCH participates in 3-hydroxyisobutyryl-CoA hydrolase deficiencyPathwayReactome R-HSA-9916722Inferred only
HIBCH participates in Amino acid metabolismPathwayWikiPathways WP3925Inferred only
HIBCH participates in Branched-chain amino acid catabolismPathwayReactome R-HSA-70895Inferred only
HIBCH participates in DiseasePathwayReactome R-HSA-1643685Inferred only
HIBCH participates in Diseases of branched-chain amino acid catabolismPathwayReactome R-HSA-9865118Inferred only
HIBCH participates in Diseases of metabolismPathwayReactome R-HSA-5668914Inferred only
HIBCH participates in Leucine isoleucine and valine metabolismPathwayWikiPathways WP4686Inferred only
HIBCH participates in MetabolismPathwayReactome R-HSA-1430728Inferred only

Show the remaining 13

HIBCH participates in Metabolism of amino acids and derivativesPathwayReactome R-HSA-71291Inferred only
CAT decreases the expression of HIBCHProteinHetionet hetionet:regulates:847-26275Inferred only
ERBB2 decreases the expression of HIBCHProteinHetionet hetionet:regulates:2064-26275Inferred only
FAS increases the expression of HIBCHProteinHetionet hetionet:regulates:355-26275Inferred only
KTN1 decreases the expression of HIBCHProteinHetionet hetionet:regulates:3895-26275Inferred only
LCK increases the expression of HIBCHProteinHetionet hetionet:regulates:3932-26275Inferred only
LTBR decreases the expression of HIBCHProteinHetionet hetionet:regulates:4055-26275Inferred only
MAST2 increases the expression of HIBCHProteinHetionet hetionet:regulates:23139-26275Inferred only
OLIG3 increases the expression of HIBCHProteinHetionet hetionet:regulates:167826-26275Inferred only
RAB11A increases the expression of HIBCHProteinHetionet hetionet:regulates:8766-26275Inferred only
SOAT2 decreases the expression of HIBCHProteinHetionet hetionet:regulates:8435-26275Inferred only
TBK1 decreases the expression of HIBCHProteinHetionet hetionet:regulates:29110-26275Inferred only
3-hydroxy-2-methylpropanoate is a substrate of HIBCHCompoundRhea RHEA:20888Inferred only

Reactions

4 reactions

MAM00799c+waterCHEBI:1146900+proton+MAM02142c
Catalysed by HIBCH
Reaction pageLeft to rightBalancedEC 3.1.2.4cytosol
Human-GEM MAR00043CC BY 4.0Reference data
MAM00785m+waterMAM00784m+CHEBI:1146900+proton
Catalysed by HIBCH
Reaction pageLeft to rightBalancedEC 3.1.2.4mitochondrial matrix

Other names

3 names

Resolves to: HIBCH

Also called: 3-hydroxyisobutyryl-coenzyme A hydrolaseHIB-CoA hydrolaseHIBYL-CoA-H

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