Overview

  • Product name

  • Description

    Rabbit polyclonal to HPS2
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

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

    Recombinant fragment corresponding to Human HPS2 aa 895-1094.
    Sequence:

    FGDKMVSIQITLNNTTDRKIENIHIGEKKLPIGMKMHVFNPIDSLEPEGS ITVSMGIDFCDSTQTASFQLCTKDDCFNVNIQPPVGELLLPVAMSEKDFK KEQGVLTGMNETSAVIIAAPQNFTPSVIFQKVVNVANVGAVPSGQDNIHR FAAKTVHSGSLMLVTVELKEGSTAQLIINTEKTVIGSVLLRELKPVLSQG


    Database link: O00203-1

  • Positive control

    • IHC-P: Human colon cancer tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab234848 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
IHC-P 1/20 - 1/200.

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

  • Paraffin-embedded human colon cancer tissue stained for HPS2 with ab234848 at 1/100 dilution in immunohistochemical analysis.

References

ab234848 has not yet been referenced specifically in any publications.

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