概述

  • 产品名称Anti-HPS2抗体
    参阅全部 HPS2 一抗
  • 描述
    山羊多克隆抗体to HPS2
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide:

    SSNSFPYNEQSGG-C

    , corresponding to amino acids 2-14 of Human HPS2 (NP_003655.3).

  • 阳性对照
    • Human Breast and Colon tissues

性能

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应用

Our Abpromise guarantee covers the use of ab118584 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
IHC-P Use a concentration of 3.75 - 7.5 µg/ml.

靶标

  • 功能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.
  • 组织特异性Ubiquitously expressed.
  • 疾病相关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.
  • 序列相似性Belongs to the adaptor complexes large subunit family.
  • 翻译后修饰Phosphorylated on serine residues.
  • 细胞定位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
  • 数据库链接
  • 别名
    • 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 图像

  • ab118584, at 7.5 µg/ml, staining HPS2 in formalin-fixed, paraffin-embedded Human Breast tissue by Immunohistochemistry.
  • ab118584, at 7.5 µg/ml, staining HPS2 in formalin-fixed, paraffin-embedded Human Colon tissue by Immunohistochemistry.

Anti-HPS2 antibody (ab118584)参考文献

ab118584 has not yet been referenced specifically in any publications.

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