VARIANT: 26275v1
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Entry
26275v1 Variant
Name
HIBCH deficiency
Gene
HIBCH
3-hydroxyisobutyryl-CoA hydrolase [KO:
K05605
]
Organism
hsa_var Human gene variants (Homo sapiens)
Variation
mutations
OmimVar:
610690
Network
nt06024
Valine, leucine and isoleucine degradation
Disease
H01349
Methacrylic aciduria
Reference
PMID:
7122152
Authors
Brown GK, Hunt SM, Scholem R, Fowler K, Grimes A, Mercer JF, Truscott RM, Cotton RG, Rogers JG, Danks DM
Title
beta-hydroxyisobutyryl coenzyme A deacylase deficiency: a defect in valine metabolism associated with physical malformations.
Journal
Pediatrics 70:532-8 (1982)
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