製品の概要

  • 製品名
    Anti-BOLA3 antibody
  • 製品の詳細
    Rabbit polyclonal to BOLA3
  • アプリケーション
    適用あり: IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat
  • 免疫原

    Recombinant fragment corresponding to BOLA3 aa 18-105.
    Sequence:

    GLPLHHRMFATQTEGELRVTQILKEKFPRATAIKVTDISGGCGAMYEIKI ESEEFKEKRTVQQHQMVNQALKEEIKEMHGLRIFTSVP


    Database link: Q53S33

  • ポジティブ・コントロール
    • Human kidney tissue.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab185339 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
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

ターゲット情報

  • 組織特異性
    Widely expressed.
  • 関連疾患
    Defects in BOLA3 are the cause of multiple mitochondrial dysfunctions syndrome type 2 (MMDS2) [MIM:614299]. A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes.
  • 配列類似性
    Belongs to the bolA/yrbA family.
  • 細胞内局在
    Secreted. Could be secreted via a non-classical export pathway.
  • Information by UniProt
  • 参照データベース
  • 別名
    • BolA-like protein 3 antibody
    • BOLA3 antibody
    • BOLA3_HUMAN antibody
    • MMDS2 antibody

画像

  • Immunohistochemistry analysis of Human kidney tissue labeling BOLA3 with ab185339 at 1/200 dilution. 

プロトコール

参考文献

ab185339 has not yet been referenced specifically in any publications.

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