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Standards Ontologies Notations
3-hydroxyisobutyryl-CoA hydrolase

Summary
Gene Symbol
  • HIBCH
Organism
Homo sapiens (human)
External Links
NCBI Gene
26275
HGNC
4908
KEGG Gene ID
hsa:26275
PubChem
26275
Alliance of Genome Resources
Annotation
Keyword
  • 3D-structure
  • Acetylation
  • Alternative splicing
  • Branched-chain amino acid catabolism
  • Disease variant
  • Hydrolase
  • Mitochondrion
  • Phosphoprotein
  • Reference proteome
  • Transit peptide
Proteins
Displaying all 2 entries
UniProt Protein Name
Q6NVY1
  • 3-hydroxyisobutyryl-coenzyme A hydrolase
A0A140VJL0
  • 3-hydroxyisobutyryl-coenzyme A hydrolase
Annotation information from UniProt.
Gene Ontology (GO)
Displaying all 2 entries
GO Term Evidence Code PMID
valine catabolic process
branched-chain amino acid catabolic process
GO Hierarchy
Gene Ontology information from NCBI Gene and UniProt.
Human Protein Atlas
ENSG00000198130

soft tissue soft tissue blood blood blood blood blood blood blood blood blood blood blood blood blood blood lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node kidney kidney adrenal gland adrenal gland breast breast duodenum stomach small intestine colon rectum appendix esophagus eye eye salivary gland nasopharynx nasopharynx tongue tonsil tonsil tonsil hair skin adipose tissue adipose tissue adipose tissue spinal cord oral mucosa lung lung heart bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus liver pancreas thyroid gland parathyroid gland parathyroid gland parathyroid gland parathyroid gland parathyroid gland spleen gallbladder ductus deferens ductus deferens seminal vesicle seminal vesicle seminal vesicle seminal vesicle testis testis epididymis epididymis brain smooth muscle urinary bladder prostate bone marrow skeletal muscle skeletal muscle skeletal muscle skeletal muscle skeletal muscle thymus thymus cartilage sole of foot sole of foot soft tissue blood blood blood blood blood blood blood blood blood blood blood blood lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lymph node lactating breast lactating breast kidney kidney adrenal gland adrenal gland duodenum stomach small intestine colon rectum appendix esophagus eye eye salivary gland salivary gland salivary gland salivary gland salivary gland salivary gland nasopharynx tongue tonsil tonsil hair skin adipose tissue adipose tissue adipose tissue adipose tissue adipose tissue adipose tissue adipose tissue spinal cord oral mucosa lung lung heart bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus bronchus liver pancreas thyroid gland parathyroid gland parathyroid gland parathyroid gland parathyroid gland parathyroid gland spleen gallbladder brain skeletal muscle skeletal muscle skeletal muscle skeletal muscle skeletal muscle thymus thymus smooth muscle urinary bladder vagina ovary ovary fallopian tube fallopian tube endometrium placenta cervix cervix bone marrow cartilage sole of foot sole of foot olfactory region cerebral cortex cerebellum medulla pons midbrain pituitary gland hypothalamus amygdala thalamus choroid plexus choroid plexus corpus callosum basal ganglia substantia nigra dorsal raphe dorsal raphe dorsal raphe dorsal raphe dorsal raphe dorsal raphe caudate hippocampus retina retina

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas
KEGG BRITE Database
Orthology
K05605
Name
3-hydroxyisobutyryl-CoA hydrolase [EC:3.1.2.4]
References
Rhea

RHEA:20888

H2O + 3-hydroxy-2-methylpropanoyl-CoA
3-hydroxy-2-methylpropanoate + H+ + CoA
Enzyme
PMID
Disease
Disease Ontology
Displaying entries 31 - 35 of 35 in total
DO ID Disease Name Source
DOID:936 brain disease
DOID:9649 congenital nystagmus
DOID:9650 pathologic nystagmus
DOID:9837 hypertropia
DOID:9923 developmental coordination disorder
The Human Phenotype Ontology
Displaying entries 21 - 30 of 42 in total
HPO ID HPO Term
HP:0001636 Tetralogy of Fallot
HP:0001942 Metabolic acidosis
HP:0002013 Vomiting
HP:0002078 Truncal ataxia
HP:0002093 Respiratory insufficiency
HP:0002119 Ventriculomegaly
HP:0002151 Increased circulating lactate concentration
HP:0002344 Progressive neurologic deterioration
HP:0002352 Leukoencephalopathy
HP:0002360 Sleep abnormality
Displaying all 2 entries
Disease ID Disease Name
ORPHA:88639
  • 3-hydroxyisobutyryl-CoA hydrolase deficiency
OMIM:250620
  • 3-hydroxyisobutyryl-CoA hydrolase deficiency
PubChem Disease
MedGen Diseases
OMIM Phenotypes
KEGG Diseases
Gene-Disease-Co-Occurrences-in-Literature (PubChem)
LIPID MAPS Gene / Proteome Database
LMPD ID
LMP004216
Gene Name
3-hydroxyisobutyryl-CoA hydrolase
Ortholog
Displaying entries 1 - 10 of 76 in total
Species Gene ID Alliance of Genome Resources Orthologous MAtrix
175766 WB:WBGene00017301
41869 FB:FBgn0038326
103176535 CALMI10683
102350181 LATCH13280
798364 ZFIN:ZDB-GENE-050327-29 DANRE45273
108266808 ICTPU31534
113581354 ELEEL18680
115160656 SALTR15520
115533062 GADMO29359
101159781 ORYLA09685
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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: August 19, 2024