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HIBCH

  • 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
  • Cell & Tissue Lysates
  • Protein Pre-coupled Magnetic Beads
  • Chicken
  • Human
  • Mouse
  • Rat
  • Zebrafish
  • E.coli
  • HEK293
  • HEK293T
  • In Vitro Cell Free System
  • Mammalian Cell
  • Wheat Germ
  • GST
  • His
  • His (Fc)
  • Avi
  • His|T7
  • Myc
  • DDK
  • Myc|DDK
  • N/A
Species Cat.# Product name Source (Host) Tag Protein Length Price
Human HIBCH-13772H Recombinant Human HIBCH, His-tagged E.coli His 1-386a.a.
Human HIBCH-244H Recombinant Human HIBCH Protein, MYC/DDK-tagged HEK293 Myc/DDK
Human HIBCH-4743H Recombinant Human HIBCH Protein, GST-tagged Wheat Germ GST
Human HIBCH-5565HCL Recombinant Human HIBCH 293 Cell Lysate HEK293 N/A
Human HIBCH-3465HF Recombinant Full Length Human HIBCH Protein, GST-tagged In Vitro Cell Free System GST 338 amino acids
Human HIBCH-3813H Recombinant Human HIBCH Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
Mouse HIBCH-7619M Recombinant Mouse HIBCH Protein Mammalian Cell His
Mouse Hibch-8168M Recombinant Mouse Hibch protein, His & T7-tagged E.coli His/T7 Met1~Gly170 (Accession # Q8QZS1)
Mouse Hibch-3392M Recombinant Mouse Hibch Protein, Myc/DDK-tagged HEK293T Myc/DDK
Mouse HIBCH-4159M Recombinant Mouse HIBCH Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Mouse HIBCH-4159M-B Recombinant Mouse HIBCH Protein Pre-coupled Magnetic Beads HEK293
Rat HIBCH-2840R Recombinant Rat HIBCH Protein Mammalian Cell His
Rat HIBCH-2495R Recombinant Rat HIBCH Protein, His (Fc)-Avi-tagged HEK293 His (Fc)-Avi
Rat HIBCH-2495R-B Recombinant Rat HIBCH Protein Pre-coupled Magnetic Beads HEK293
Zebrafish HIBCH-2247Z Recombinant Zebrafish HIBCH Mammalian Cell His
Chicken HIBCH-2903C Recombinant Chicken HIBCH Mammalian Cell His
  • Involved Pathway
  • Protein Function
  • Interacting Protein
  • HIBCH Related Articles

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
Valine, leucine and isoleucine degradationDLDH;ALDH1B1;MCEE;ALDH7A1;AOX3;ALDH3A2A;HADHAA;OXCT2;AOX2P
beta-Alanine metabolismHADHAA;ALDH2.2;HIBCH;CARNS1;SRM;ALDH9A1B;UPB1;ALDH9A1;CNDP2
Propanoate metabolismMLYCD;SUCLA2;DLDH;ACAT1;HADHAB;HADHAA;ACADM;ALDH6A1;PCCB
Metabolic pathwaysGALNTL2;UGT8;AFMID;SCP2A;ENO1;PLCD3A;ATP5J2;CYP4A10;NAGS
Carbon metabolismPRPS1A;GPI1;HADHAB;MDH2;PDHA2;PFKMB;ALDOAA;FBP1;PRPS2

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
3-hydroxyisobutyryl-CoA hydrolase activity
hydro-lyase activityCAR1;HIBCH;L3HYPDH;CA1

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

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).
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