概述

  • 产品名称Anti-HIBCH抗体
    参阅全部 HIBCH 一抗
  • 描述
    兔多克隆抗体to HIBCH
  • 经测试应用适用于: IHC-P, WB, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • HepG2 mitochondrial lysate

性能

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应用

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.

应用 Ab评论 说明
IHC-P 1/50 - 1/100.
WB 1/50 - 1/100. Predicted molecular weight: 43 kDa.
ELISA 1/1000.

靶标

  • 功能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.
  • 组织特异性Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • 通路Amino-acid degradation; L-valine degradation.
  • 疾病相关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.
  • 序列相似性Belongs to the enoyl-CoA hydratase/isomerase family.
  • 细胞定位Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • 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)参考文献

ab82413 has not yet been referenced specifically in any publications.

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