概述

  • 产品名称Anti-MCCC2抗体
    参阅全部 MCCC2 一抗
  • 描述
    兔多克隆抗体to MCCC2
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    A KLH conjugated synthetic peptide selected from the Center region of Human MCCC2.

  • 阳性对照
    • 293 cell line lysates and mouse liver tissue lysates

性能

应用

Our Abpromise guarantee covers the use of ab91414 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
ELISA
  • 应用说明ELISA: 1/1000.
    WB: 1/100 - 1/500. Predicted molecular weight: 61 kDa.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 通路Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
    • 疾病相关Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) [MIM:210210]. MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.
    • 序列相似性Belongs to the AccD/PCCB family.
      Contains 1 carboxyltransferase domain.
    • 细胞定位Mitochondrion matrix.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 3 methylcrotonyl CoA carboxylase 2 antibody
      • 3 methylcrotonyl CoA carboxylase non biotin containing subunit antibody
      • 3 methylcrotonyl CoA:carbon dioxide ligase subunit beta antibody
      • 3-methylcrotonyl-CoA carboxylase 2 antibody
      • 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody
      • 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody
      • Biotin carboxylase antibody
      • MCCase subunit beta antibody
      • MCCB antibody
      • MCCB_HUMAN antibody
      • MCCC 2 antibody
      • Mccc2 antibody
      • Methylcrotonoyl CoA carboxylase 2 (beta) antibody
      • Methylcrotonoyl CoA carboxylase beta chain mitochondrial antibody
      • Methylcrotonoyl Coenzyme A carboxylase 2 (beta) antibody
      • Methylcrotonoyl-CoA carboxylase beta chain antibody
      • mitochondrial antibody
      • Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase antibody
      see all

    Anti-MCCC2 antibody 图像

    • Anti-MCCC2 antibody (ab91414) at 1/100 dilution + 293 cell line lysates at 35 µg

      Predicted band size : 61 kDa
    • Anti-MCCC2 antibody (ab91414) at 1/100 dilution + mouse liver tissue lysates at 35 µg

      Predicted band size : 61 kDa

    Anti-MCCC2 antibody (ab91414)参考文献

    ab91414 has not yet been referenced specifically in any publications.

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