製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号Q6NVY1
    • 生物種Human
    • 配列MGSSHHHHHH 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
    • 分子量42 kDa including tags
    • 領域33 to 386
    • タグHis tag N-Terminus

特性

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.

  • アプリケーション

    SDS-PAGE

    Mass Spectrometry

  • 質量分析
    MALDI-TOF
  • 精製度> 90 % SDS-PAGE.
    ab124585 is purified using conventional chromatography techniques.
  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能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.
  • 組織特異性Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • パスウェイAmino-acid degradation; L-valine degradation.
  • 関連疾患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.
  • 配列類似性Belongs to the enoyl-CoA hydratase/isomerase family.
  • 細胞内局在Mitochondrion.
  • Information by UniProt

Recombinant Human HIBCH protein 画像

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

Recombinant Human HIBCH protein (ab124585) 使用論文

ab124585 has not yet been referenced specifically in any publications.

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