Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionQ6NVY1
    • SpeciesHuman
    • SequenceMGSSHHHHHH SSGLVPRGSH MGSHMDAAEE VLLEKKGCTG VITLNRPKFL NALTLNMIRQ IYPQLKKWEQ DPETFLIIIK GAGGKAFCAG GDIRVISEAE KAKQKIAPVF FREEYMLNNA VGSCQKPYVA LIHGITMGGG VGLSVHGQFR VATEKCLFAM PETAIGLFPD VGGGYFLPRL QGKLGYFLAL TGFRLKGRDV YRAGIATHFV DSEKLAMLEE DLLALKSPSK ENIASVLENY HTESKIDRDK SFILEEHMDK INSCFSANTV EEIIENLQQD GSSFALEQLK VINKMSPTSL KITLRQLMEG SSKTLQEVLT MEYRLSQACM RGHDFHEGVR AVLIDKDQSP KWKPADLKEV TEEDLNNHFK SLGSSDLKF
    • Molecular weight42 kDa including tags
    • Amino acids33 to 386
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab124585 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Mass Spectrometry

  • Mass spectrometry
    MALDI-TOF
  • Purity> 90 % SDS-PAGE.
    ab124585 is purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 1.17% Sodium chloride

General Info

  • Alternative names
    • 3 hydroxyisobutyryl Coenzyme A hydrolase
    • 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial
    • 3-hydroxyisobutyryl-CoA hydrolase
    • 3-hydroxyisobutyryl-coenzyme A hydrolase
    • BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE
    • HIB CoA hydrolase
    • HIB-CoA hydrolase
    • HIBCH
    • HIBCH_HUMAN
    • HIBCoA hydrolase
    • HIBYL CoA H
    • HIBYL CoAH
    • HIBYL-CoA-H
    • HIBYLCoA H
    • HIBYLCoAH
    • mitochondrial
    see all
  • 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

Recombinant Human HIBCH protein images

  • 15% SDS-PAGE showing ab124585 at approximately 42.1kDa (3µg).

References for Recombinant Human HIBCH protein (ab124585)

ab124585 has not yet been referenced specifically in any publications.

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