製品の概要

  • 製品名Anti-MCCC2 antibody
    MCCC2 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to MCCC2
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

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

  • ポジティブ・コントロール
    • Human liver tissue, MCCC2 transfected 293T cell lysate

製品の特性

関連製品

アプリケーション

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.

アプリケーション Abreviews 特記事項
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.

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

Anti-MCCC2 antibody 画像

  • 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) 使用論文

ab118370 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"