製品の概要

  • 製品名Anti-HIBCH antibody
    HIBCH 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to HIBCH
  • アプリケーション適用あり: IHC-P, WB, ELISAmore details
  • 種交差性
    交差種: Human
  • 免疫原

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

  • ポジティブ・コントロール
    • HepG2 mitochondrial lysate

製品の特性

関連製品

アプリケーション

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.

アプリケーション Abreviews 特記事項
IHC-P 1/50 - 1/100.
WB 1/50 - 1/100. Predicted molecular weight: 43 kDa.
ELISA 1/1000.

ターゲット情報

  • 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
  • 参照データベース
  • 別名
    • 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 画像

  • 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).

Anti-HIBCH antibody (ab82413) 使用論文

ab82413 has not yet been referenced specifically in any publications.

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