概述

  • 产品名称
  • 描述
    山羊多克隆抗体to HPS3
  • 经测试应用
    适用于: WB, ELISAmore details
  • 种属反应性

    预测可用于: Human
  • 免疫原

    Synthetic peptide:

    PYLLYCSRKKPLT

    , corresponding to C terminal amino acids 992-1004 of Human HPS3.

  • 阳性对照
    • A431 cell lysate.

性能

  • 形式
    Liquid
  • 存放说明
    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • 存储溶液
    Preservative: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline. pH 7.3
  • Concentration information loading...
  • 纯度
    Immunogen affinity purified
  • 纯化说明
    Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • 克隆
    多克隆
  • 同种型
    IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab19047 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
WB Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 113 kDa (predicted molecular weight: 113 kDa).
ELISA 1/32000.

靶标

  • 相关性
    HPS3 is involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa) mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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.
  • 细胞定位
    Cytoplasmic
  • 数据库链接
  • 别名
    • BLOC2S1 antibody
    • Cocoa antibody
    • DKFZp686F0413 antibody
    • FLJ22704 antibody
    • Hermansky Pudlak syndrome 3 antibody
    • Hermansky Pudlak syndrome 3 protein antibody
    • HPS3, biogenesis of lysosomal organelles complex 2 subunit 1 antibody
    • SUTAL antibody
    see all

图片



  • Predicted band size : 113 kDa


    ab19047 (1µg/ml) staining of A431 lysate (35µg protein in RIPA buffer) approx. 113kDa. Primary incubation was 1 hour. Detected by chemiluminescence.

实验方案

文献

This product has been referenced in:
  • Li K  et al. HPS6 interacts with dynactin p150Glued to mediate retrograde trafficking and maturation of lysosomes. J Cell Sci 127:4574-88 (2014). Read more (PubMed: 25189619) »

See 1 Publication for this product

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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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