Overview

  • Product name

    Anti-HPS2 antibody - C-terminal
    See all HPS2 primary antibodies
  • Description

    Rabbit polyclonal to HPS2 - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Cow, Dog
  • Immunogen

    Recombinant fragment corresponding to Human HPS2 aa 944-1094 (C terminal).
    Sequence:

    SITVSMGIDFCDSTQTASFQLCTKDDCFNVNIQPPVGELLLPVAMSEKDF KKEQGVLTGMNETSAVIIAAPQNFTPSVIFQKVVNVANVGAVPSGQDNIH RFAAKTVHSGSLMLVTVELKEGSTAQLIINTEKTVIGSVLLRELKPVLSQ G


    Database link: O00203

  • Positive control

    • Human fetal liver lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab197155 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/500 - 1/1000. Predicted molecular weight: 121 kDa.

Target

  • Function

    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.
  • Tissue specificity

    Ubiquitously expressed.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the adaptor complexes large subunit family.
  • Post-translational
    modifications

    Phosphorylated on serine residues.
  • Cellular localization

    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
  • Database links

  • Alternative names

    • 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

Images

  • Anti-HPS2 antibody - C-terminal (ab197155) at 1/1000 dilution + Human fetal liver lysate

    Predicted band size: 121 kDa

References

ab197155 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab197155.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up