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- Table of Contents
Facts about 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial.
Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
| Human | |
|---|---|
| Gene Name: | HIBCH |
| Uniprot: | Q6NVY1 |
| Entrez: | 26275 |

| Belongs to: |
|---|
| enoyl-CoA hydratase/isomerase family |

3-hydroxyisobutyryl-CoA hydrolase; EC 3.1.2.4,3-hydroxyisobutyryl-coenzyme A hydrolase3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIB-CoA hydrolase; HIBYLCOAH; HIBYL-CoA-H
Mass (kDA):
43.482 kDA

| Human | |
|---|---|
| Location: | 2q32.2 |
| Sequence: | 2; NC_000002.12 (190203920..190319826, complement) |
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
Mitochondrion.





PMID: 8824301 by Hawes J.W., et al. Primary structure and tissue-specific expression of human beta- hydroxyisobutyryl-coenzyme A hydrolase.
PMID: 17160907 by Loupatty F.J., et al. Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.