Overview

Properties

Associated products

Applications

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.

Application Abreviews Notes
IHC-P Use a concentration of 3.75 - 7.5 µg/ml.

Target

  • FunctionSubunit 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 specificityUbiquitously expressed.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the adaptor complexes large subunit family.
  • Post-translational
    modifications
    Phosphorylated on serine residues.
  • Cellular localizationGolgi 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

Anti-HPS2 antibody images

  • 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.

References for Anti-HPS2 antibody (ab118584)

ab118584 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"