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.
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Anti-HIBCH antibodies

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

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Target: HIBCH
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human, Dog, Rat
Clone: CBFYH-1072
Application*: WB, IF, F
Datasheet SDS

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

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Target: HIBCH
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Dog, Monkey, Rat
Clone: CBFYH-1071
Application*: F, IF, WB
Datasheet SDS

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

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Target: HIBCH
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Monkey, Rat
Clone: CBFYH-1070
Application*: F, IF, WB
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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