Overview

  • Product nameAnti-HIBCH antibody
    See all HIBCH primary antibodies
  • Description
    Rabbit polyclonal to HIBCH
  • Tested applicationsSuitable for: IHC-P, WB, ELISAmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    A KLH conjugated synthetic peptide selected from the Center region of human HIBCH.

  • Positive control
    • HepG2 mitochondrial lysate

Properties

Associated products

Applications

Our Abpromise guarantee covers the use of ab82413 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/50 - 1/100.
WB 1/50 - 1/100. Predicted molecular weight: 43 kDa.
ELISA 1/1000.

Target

  • FunctionHydrolyzes 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.
  • Tissue specificityHighly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • PathwayAmino-acid degradation; L-valine degradation.
  • Involvement in diseaseDefects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life.
  • Sequence similaritiesBelongs to the enoyl-CoA hydratase/isomerase family.
  • Cellular localizationMitochondrion.
  • Information by UniProt
  • Database links
  • Alternative names
    • 3 hydroxyisobutyryl Coenzyme A hydrolase antibody
    • 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial antibody
    • 3-hydroxyisobutyryl-CoA hydrolase antibody
    • 3-hydroxyisobutyryl-coenzyme A hydrolase antibody
    • BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE antibody
    • HIB CoA hydrolase antibody
    • HIB-CoA hydrolase antibody
    • HIBCH antibody
    • HIBCH_HUMAN antibody
    • HIBCoA hydrolase antibody
    • HIBYL CoA H antibody
    • HIBYL CoAH antibody
    • HIBYL-CoA-H antibody
    • HIBYLCoA H antibody
    • HIBYLCoAH antibody
    • mitochondrial antibody
    see all

Anti-HIBCH antibody images

  • Anti-HIBCH antibody (ab82413) at 1/50 dilution + HepG2 cell line lysates at 12.5 µg

    Predicted band size : 43 kDa
  • ab82413 at 1/100 dilution staining HIBCH in human hepatocarcinoma tissue section by Immunohistochemistry (Formalin/ PFA fixed paraffin embedded tissue sections).

References for Anti-HIBCH antibody (ab82413)

ab82413 has not yet been referenced specifically in any publications.

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