概述

  • 产品名称
  • 描述
    兔多克隆抗体to BCKDHB
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within N terminal amino acids 44-74 of Human BCKDHB (NP_000047.1).

  • 阳性对照
    • HeLa cell line lysate

性能

应用

Our Abpromise guarantee covers the use of ab107398 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
WB 1/100 - 1/500. Predicted molecular weight: 43 kDa.

靶标

  • 功能
    The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
  • 疾病相关
    Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B) [MIM:248600]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.
  • 细胞定位
    Mitochondrion matrix.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 2 oxoisovalerate dehydrogenase subunit beta mitochondrial antibody
    • 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial antibody
    • BCKDE1B antibody
    • BCKDH E1-beta antibody
    • BCKDHB antibody
    • Branched chain alpha ketoacid dehydrogenase E1 beta subunit antibody
    • Branched chain keto acid dehydrogenase E1 beta polypeptide antibody
    • Branched-chain alpha-keto acid dehydrogenase E1 component beta chain antibody
    • dJ279A18.1 antibody
    • E1B antibody
    • E1b beta subunit of the branched chain complex antibody
    • FLJ17880 antibody
    • ODBB_HUMAN antibody
    • RP1-279A18.1 antibody
    see all

图片

  • Anti-BCKDHB antibody (ab107398) at 1/100 dilution + HeLa cell line lysate at 35 µg

    Predicted band size : 43 kDa

实验方案

文献

ab107398 has not yet been referenced specifically in any publications.

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