HIBCH
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Official Full Name
3-hydroxyisobutyryl-CoA hydrolase -
Overview
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. -
Synonyms
HIBCH;3-hydroxyisobutyryl-CoA hydrolase;3 hydroxyisobutyryl Coenzyme A hydrolase;3-hydroxyisobutyryl-CoA hydrolase, mitochondrial;HIBYL-CoA-H;HIB-CoA hydrolase;3-hydroxyisobutyryl-Coenzyme A hydrolase;HIBYLCOAH
Recombinant Proteins
- Human
- Zebrafish
- Rat
- Chicken
- Mouse
- E.coli
- Mammalian Cell
- HEK293
- Wheat Germ
- HEK293T
- In Vitro Cell Free System
- His
- Myc&DDK
- GST
- His&T7
- Non
- His&Fc&Avi
Involved Pathway
HIBCH involved in several pathways and played different roles in them. We selected most pathways HIBCH participated on our site, such as Valine, leucine and isoleucine degradation,beta-Alanine metabolism,Propanoate metabolism, which may be useful for your reference. Also, other proteins which involved in the same pathway with HIBCH were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| beta-Alanine metabolism | ALDH2.1,ALDH2,ALDH9A1B,ALDH3B1,AOC3,ALDH1B1,SMOX,ALDH3A2,CNDP1,UPB1 |
| Propanoate metabolism | ECHDC1,LDHAL6B,ACSS1,DLDH,MUT,ACADM,LDHBA,LDHC,BCKDHA,ACACA |
| Metabolic pathways | POLE,UGT1A7,NT5C1B-RDH14,ATP5I,POLG,PDHB,LPIN3,GNPDA2,SC4MOL,LTA4H |
| Carbon metabolism | ACSS2L,RPE,GPI1,DAK,PGAM1,HKDC1,GOT1,ALDOB,PGAM2,SDHB |
| Valine, leucine and isoleucine degradation | IVD,OXCT1B,ACAA1A,HMGCLL1,ALDH9A1B,BCAT1,ACAA1,OXCT2B,ACADM,ALDH2.1 |
Protein Function
HIBCH has several biochemical functions, for example, 3-hydroxyisobutyryl-CoA hydrolase activity,hydro-lyase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by HIBCH itself. We selected most functions HIBCH had, and list some proteins which have the same functions with HIBCH. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| hydro-lyase activity | CAR1,CA1,L3HYPDH |
Interacting Protein
HIBCH has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with HIBCH here. Most of them are supplied by our site. Hope this information will be useful for your research of HIBCH.
GRB2;H2AFX
Resources
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References
- Soler-Alfonso, C; Enns, GM; et al. Identification of HIBCH Gene Mutations Causing Autosomal Recessive Leigh Syndrome: A Gene Involved in Valine Metabolism. PEDIATRIC NEUROLOGY 52:361-365(2015).
- Reuter, MS; Sass, JO; et al. HIBCH Deficiency in a Patient with Phenotypic Characteristics of Mitochondrial Disorders. AMERICAN JOURNAL OF MEDICAL GENETICS PART A 164A:3162-3169(2014).
