概述

  • 产品名称Anti-MCCC2抗体
    参阅全部 MCCC2 一抗
  • 描述
    小鼠单克隆抗体to MCCC2
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant fragment, corresponding to amino acids 456-564 of Human MCCC2 (NP_071415) with a proprietary tag.

  • 阳性对照
    • Human liver tissue, MCCC2 transfected 293T cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab118370 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 Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 61 kDa.
IHC-P Use a concentration of 3 µg/ml. Antigen retrieval is not essential but may optimise staining. Place sample in 1X citrate buffer (pH 6.0) in pressure cooker under 125oC for 4min and under 90oC for 45min.

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

  • All lanes : Anti-MCCC2 antibody (ab118370) at 5 µg/ml

    Lane 1 : MCCC2 transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 61 kDa
  • Formalin-fixed, paraffin-embedded Human liver tissue labelled with ab118370 at 3 µg/ml
  • Anti-MCCC2 antibody (ab118370) at 5 µg/ml + Recombinant Protein (immunogen) at 0.2 µg

    Predicted band size : 61 kDa

Anti-MCCC2 antibody (ab118370)参考文献

ab118370 has not yet been referenced specifically in any publications.

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