| 1308392 | Hibch | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (ortholog); INVOLVED IN L-valine catabolic process (ortholog); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathw ay; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrion (ortholog); INTERACTS WITH 3-chloropropane-1,2-diol; bisphenol A; Brodifacoum | 9 | 56082082 | 56161796 | Rat | 163 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 1318134 | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010] | 2 | 190189735 | 190319826 | Human | 400 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1318135 | Hibch | 3-hydroxyisobutyryl-Coenzyme A hydrolase | Predicted to enable 3-hydroxyisobutyryl-CoA hydrolase activity. Predicted to be involved in valine catabolic process. Located in mitochondrion. Human ortholog(s) of this gene implicated in 3-hydroxyisobutryl-CoA hydrolase deficiency. Orthologous to human HIBCH ( 3-hydroxyisobutyryl-CoA hydrolase). [provided by Alliance of Genome Resources, Apr 2025] | 1 | 52884197 | 52960145 | Mouse | 168 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 9023279 | Hibch | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (ortholog); INVOLVED IN L-valine catabolic process (ortholog); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathw ay; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrion (ortholog) | NW_004955403 | 8752339 | 8796693 | Chinchilla | 25 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11746539 | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (ortholog) | 2B | 77510938 | 77652911 | Bonobo | 34 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12134376 | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrion (inferred) | 37 | 841385 | 943972 | Dog | 36 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12636818 | Hibch | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrion (inferred) | NW_004936506 | 7811624 | 7882861 | Squirrel | 37 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626073645 | Hibch | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (ortholog); INVOLVED IN L-valine catabolic process (ortholog); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathw ay; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrion (ortholog) | | | | Black Rat | 25 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18910157 | Hibch | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (ortholog) | | | | Naked Mole-Rat | 34 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18749904 | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrion (inferred) | | | | Green Monkey | 37 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14102511 | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | ENCODES a protein that exhibits 3-hydroxyisobutyryl-CoA hydrolase activity (inferred); hydrolase activity (inferred); INVOLVED IN branched-chain amino acid catabolic process (inferred); L-valine catabolic process (inferred); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3- hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-hydroxyisobutryl-CoA hydrolase deficiency (ortholog); genetic disease (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrion (inferred); INTERACTS WITH deoxynivalenol | | | | Pig | 38 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155241640 | hibch | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 155241641 | hibch.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 155241756 | hibch.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 16558537 | AC116359.1 | 3-hydroxyisobutyryl-Coenzyme A hydrolase (HIBCH) pseudogene | | 5 | 2787251 | 2788608 | Human | | name | gene, pseudo, INFERRED [RefSeq] |