Gene Symbol |
HIBCH
|
Entrez Gene |
26275
|
Alt Symbol |
HIBYLCOAH
|
Species |
Human
|
Gene Type |
protein-coding
|
Description |
3-hydroxyisobutyryl-CoA hydrolase
|
Other Description |
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial|3-hydroxyisobutyryl-Coenzyme A hydrolase|HIB-CoA hydrolase|HIBYL-CoA-H
|
Swissprots |
Q53RF7 Q53GF2 Q53GA8 Q53TC6 D3DPI4 Q6NVY1 Q92931 Q9BS94
|
Accessions |
AAX93234 AAY24178 EAX10873 EAX10874 EAX10875 EAX10876 EAX10877 Q6NVY1 AI767349 AK126890 AK222979 BAD96699 AK223023 BAD96743 AK308042 AK313651 BC005190 AAH05190 BC067822 AAH67822 BI764401 BP193393 CA448468 CA453768 DB034438 U66669 AAC52114 XM_011510953 XP_011509255 XM_011510954 XP_011509256 XR_922903 NM_014362 NP_055177 NM_198047 NP_932164
|
Function |
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. {ECO:0000269|PubMed:8824301}.
|
Subcellular Location |
Mitochondrion {ECO:0000250}.
|
Tissue Specificity |
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. {ECO:0000269|PubMed:8824301}.
|
Top Pathways |
Carbon metabolism, Valine, leucine and isoleucine degradation, beta-Alanine metabolism, Propanoate metabolism
|