HIBCH (3-hydroxyisobutyryl-CoA hydrolase)

2014-11-01  

Identity

HGNC
LOCATION
2q32.2
LOCUSID
ALIAS
HIBYLCOAH
FUSION GENES

Other Information

Locus ID:

NCBI: 26275
MIM: 610690
HGNC: 4908
Ensembl: ENSG00000198130

Variants:

dbSNP: 26275
ClinVar: 26275
TCGA: ENSG00000198130
COSMIC: HIBCH

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000198130ENST00000359678Q6NVY1
ENSG00000198130ENST00000359678A0A140VJL0
ENSG00000198130ENST00000392332Q6NVY1
ENSG00000198130ENST00000392333H7BYI7
ENSG00000198130ENST00000399855H7C126
ENSG00000198130ENST00000409820F8W8A6
ENSG00000198130ENST00000409934B8ZZZ0
ENSG00000198130ENST00000410045B9A058
ENSG00000198130ENST00000414928H7C400
ENSG00000198130ENST00000416732H7C1A5

Expression (GTEx)

0
5
10
15
20
25
30
35
40

Pathways

PathwaySourceExternal ID
Valine, leucine and isoleucine degradationKEGGko00280
beta-Alanine metabolismKEGGko00410
Propanoate metabolismKEGGko00640
Valine, leucine and isoleucine degradationKEGGhsa00280
beta-Alanine metabolismKEGGhsa00410
Propanoate metabolismKEGGhsa00640
Metabolic pathwaysKEGGhsa01100
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoAKEGGhsa_M00013
Malonate semialdehyde pathway, propanoyl-CoA => acetyl-CoAKEGGM00013
Carbon metabolismKEGGhsa01200
Carbon metabolismKEGGko01200
MetabolismREACTOMER-HSA-1430728
Metabolism of amino acids and derivativesREACTOMER-HSA-71291
Branched-chain amino acid catabolismREACTOMER-HSA-70895

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
326770932021Delineating the neurological phenotype in children with defects in the ECHS1 or HIBCH gene.11
326770932021Delineating the neurological phenotype in children with defects in the ECHS1 or HIBCH gene.11
312031922019A genetic epidemiological study in British adults and older adults shows a high heritability of the combined indicator of vitamin B(12) status (cB(12)) and connects B(12) status with utilization of mitochondrial substrates and energy metabolism.5
314097692019Targeting HIBCH to reprogram valine metabolism for the treatment of colorectal cancer.13
312031922019A genetic epidemiological study in British adults and older adults shows a high heritability of the combined indicator of vitamin B(12) status (cB(12)) and connects B(12) status with utilization of mitochondrial substrates and energy metabolism.5
314097692019Targeting HIBCH to reprogram valine metabolism for the treatment of colorectal cancer.13
271325952016A Common Polymorphism in HIBCH Influences Methylmalonic Acid Concentrations in Blood Independently of Cobalamin.28
274008042016A movement disorder with dystonia and ataxia caused by a mutation in the HIBCH gene.15
271325952016A Common Polymorphism in HIBCH Influences Methylmalonic Acid Concentrations in Blood Independently of Cobalamin.28
274008042016A movement disorder with dystonia and ataxia caused by a mutation in the HIBCH gene.15
242994522013HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase.35
242994522013HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase.35
208776242010Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.20
208776242010Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.20
171609072007Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.38

Citation

Dessen P

HIBCH (3-hydroxyisobutyryl-CoA hydrolase)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/64263/hibch-(3-hydroxyisobutyryl-coa-hydrolase)