Overview

  • Product name
  • Description
    Rabbit polyclonal to HIBCH
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 1-288 of Human HIBCH (UniProt: Q6NVY1).

  • Positive control
    • 293T, HepG2, Molt4 whole cell lysates; A431 cells; Hepatoma tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab153826 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
WB 1/500 - 1/3000. Predicted molecular weight: 43 kDa.
IHC-P 1/100 - 1/1000.
ICC/IF 1/100 - 1/1000.

Target

  • 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.
  • Tissue specificity
    Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • Pathway
    Amino-acid degradation; L-valine degradation.
  • Involvement in disease
    Defects 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 similarities
    Belongs to the enoyl-CoA hydratase/isomerase family.
  • Cellular localization
    Mitochondrion.
  • 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

Images

  • All lanes : Anti-HIBCH antibody (ab153826) at 1/1000 dilution

    Lane 1 : 293T whole cell lysate
    Lane 2 : HepG2 whole cell lysate
    Lane 3 : Molt4 whole cell lysate

    Lysates/proteins at 30 µg per lane.

    Predicted band size: 43 kDa



    10% SDS PAGE
  • Immunofluorescence analysis of methanol fixed A431 cells labeling HIBCH with ab153826 at 1/200. Lower panel shows costaining with Hoechst 33342.
  • Immunohistochemical analysis of paraffin embedded Human hepatoma tissue labeling HIBCH with ab153826 at 1/500.

References

ab153826 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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