製品の概要

  • 製品名
  • 製品の詳細
    Rabbit polyclonal to MCCC2
  • アプリケーション
    適用あり: WB, ELISAmore details
  • 種交差性
    交差種: Mouse, Human
  • 免疫原

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

  • ポジティブ・コントロール
    • 293 cell line lysates and mouse liver tissue lysates

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB
ELISA
  • 追加情報
    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.
  • ターゲット情報

    • パスウェイ
      Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
    • 関連疾患
      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.
    • 配列類似性
      Belongs to the AccD/PCCB family.
      Contains 1 carboxyltransferase domain.
    • 細胞内局在
      Mitochondrion matrix.
    • Information by UniProt
    • 参照データベース
    • 別名
      • 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

    画像

    • 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

    プロトコール

    参考文献

    ab91414 has not yet been referenced specifically in any publications.

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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