製品の概要

  • 製品名Anti-HPS2 antibody
    HPS2 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to HPS2
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Rat
    交差が予測される動物種: Mouse, Horse, Cow, Cat, Pig
  • 免疫原

    Synthetic peptide corresponding to a region within N-terminal amino acids 1-50 (MSSNSFAYNE QSGGGEAAEL GQEATSTISS SGAFGLFSSD WKKNEDLKQM) of Rat HPS2 (EDM10075).

  • ポジティブ・コントロール
    • Rat liver lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab111506 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 µg/ml. Predicted molecular weight: 121 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

ターゲット情報

  • 機能Subunit of non-clathrin- and clathrin-associated adaptor protein complex 3 that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. AP-3 appears to be involved in the sorting of a subset of transmembrane proteins targeted to lysosomes and lysosome-related organelles.
  • 組織特異性Ubiquitously expressed.
  • 関連疾患Defects in AP3B1 are the cause of Hermansky-Pudlak syndrome type 2 (HPS2) [MIM:608233]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. HPS2 differs from the other forms of HPS in that it includes immunodeficiency in its phenotype and patients with HPS2 have an increased susceptibility to infections.
  • 配列類似性Belongs to the adaptor complexes large subunit family.
  • 翻訳後修飾Phosphorylated on serine residues.
  • 細胞内局在Golgi apparatus. Cytoplasmic vesicle > clathrin-coated vesicle membrane. Golgi apparatus. Component of the coat surrounding the cytoplasmic face of coated vesicles located at the Golgi complex.
  • Information by UniProt
  • 参照データベース
  • 別名
    • Adapter related protein complex 3 beta 1 subunit antibody
    • Adapter-related protein complex 3 subunit beta-1 antibody
    • Adaptor protein complex AP-3 subunit beta-1 antibody
    • Adaptor protein complex AP3 beta1 subunit antibody
    • ADTB3 antibody
    • ADTB3A antibody
    • AP-3 complex subunit beta-1 antibody
    • AP3 complex beta1 subunit antibody
    • AP3B1 antibody
    • AP3B1_HUMAN antibody
    • Beta-3A-adaptin antibody
    • Beta3A adaptin antibody
    • Clathrin assembly protein complex 3 beta 1 large chain antibody
    • Clathrin assembly protein complex 3 beta-1 large chain antibody
    • HPS antibody
    • PE antibody
    see all

Anti-HPS2 antibody 画像

  • Anti-HPS2 antibody (ab111506) at 1 µg/ml + Rat liver lysate at 10 µg

    Predicted band size : 121 kDa

Anti-HPS2 antibody (ab111506) 使用論文

ab111506 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"