HIBCH

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.

Full Name

3-Hydroxyisobutyryl-CoA Hydrolase

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.

Biological Process

Branched-chain amino acid catabolic process Source: Reactome
Valine catabolic process Source: GO_Central

Cellular Location

Mitochondrion

Involvement in disease

3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD):
An autosomal recessive inborn error of valine metabolism. It causes severely delayed psychomotor development, neurodegeneration, increased lactic acid, and brain lesions in the basal ganglia.

Anti-HIBCH antibodies

Mouse Anti-HIBCH Recombinant Antibody (CBFYH-1072) (CBMAB-H2015-FY)

Datasheet

SDS

Target: HIBCH

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human, Dog, Rat

Clone: CBFYH-1072

Application*: WB, IF, F

Mouse Anti-HIBCH Recombinant Antibody (CBFYH-1071) (CBMAB-H2014-FY)

Datasheet

SDS

Target: HIBCH

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human, Dog, Monkey, Rat

Clone: CBFYH-1071

Application*: F, IF, WB

Mouse Anti-HIBCH Recombinant Antibody (CBFYH-1070) (CBMAB-H2013-FY)

Datasheet

SDS

Target: HIBCH

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Dog, Monkey, Rat

Clone: CBFYH-1070

Application*: F, IF, WB

For Research Use Only. Not For Clinical Use.

(P): Predicted

* Abbreviations

IFImmunofluorescence

IHImmunohistochemistry

IPImmunoprecipitation

WBWestern Blot

EELISA

MMicroarray

CIChromatin Immunoprecipitation

FFlow Cytometry

FNFunction Assay

IDImmunodiffusion

RRadioimmunoassay

TCTissue Culture

GSGel Supershift

NNeutralization

BBlocking

AActivation

IInhibition

DDepletion

ESELISpot

DBDot Blot

MCMass Cytometry/CyTOF

CTCytotoxicity

SStimulation

AGAgonist

APApoptosis

IMImmunomicroscopy

BABioassay

CSCostimulation

EMElectron Microscopy

IEImmunoelectrophoresis

PAPeptide Array

ICImmunocytochemistry

PEPeptide ELISA

MDMeDIP

SHIn situ hybridization

IAEnzyme Immunoassay

SEsandwich ELISA

PLProximity Ligation Assay

ECELISA(Cap)

EDELISA(Det)

BIBioimaging

IOImmunoassay

LFLateral Flow Immunoassay

LALuminex Assay

CImmunohistochemistry-Frozen Sections

PImmunohistologyp-Paraffin Sections

ISIntracellular Staining for Flow Cytometry

MSElectrophoretic Mobility Shift Assay

RIRNA Binding Protein Immunoprecipitation (RIP)