Overview

  • Product name

  • Description

    Rabbit polyclonal to HIBCH
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse
    Predicted to work with: Rat
  • Immunogen

    Recombinant fragment (His-T7-tag) corresponding to Mouse HIBCH aa 1-170. (Expressed in E.coli).
    Sequence:

    MGQPYAWRLLSRVSSFRRASVILQHLRMSMHTEAAEVLLERRGCGGVITL NRPKFLNALSLNMIRQIYPQLKTWEQDPDTFLIIIKGAGGKAFCAGGDIK ALSEAKKARQNLTQDLFREEYILNNAIASCQKPYVALIDGITMGGGVGLS VHGQFRVATERSLFAMPETG


    Database link: Q8QZS1

  • Positive control

    • IHC-P: Mouse kidney tissue. WB: Mouse lung lysate; Recombinant mouse HIBCH protein.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    ab231820 was purified by antigen-specific affinity chromatography followed by Protein A affinity chromatography.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab231820 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 Use a concentration of 0.2 - 2 µg/ml. Predicted molecular weight: 43 kDa.
IHC-P Use a concentration of 5 - 20 µg/ml.

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

  • Formalin-fixed, paraffin-embedded mouse kidney tissue stained for HIBCH using ab231820 at 20 µg/ml in immunohistochemical analysis. DAB staining.

  • Anti-HIBCH antibody (ab231820) at 2 µg/ml + Recombinant mouse HIBCH protein

    Predicted band size: 43 kDa

  • Anti-HIBCH antibody (ab231820) at 1 µg/ml + Mouse lung lysate

    Predicted band size: 43 kDa

References

ab231820 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab231820.
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