Anti-HPS1 Antibody

Raised in rabbit hosts as an unconjugated polyclonal IgG reagent, this Antibodies.com antibody recognizes the HPS1 protein, which assembles with HPS4 to form the biogenesis of lysosome-related organelles complex 3 (BLOC-3). BLOC-3 governs the formation of specialized lysosome-related organelles (LROs), including melanosomes that distribute melanin in melanocytes and platelet dense granules that mediate clot formation, and it does so through longin-domain interactions with the small GTPases RAB32/38 and RAB9A.

Validated for Western blot, immunohistochemistry, and immunofluorescence, the antibody detects endogenous HPS1 in human, mouse, and rat samples, supporting comparative work on melanosomal trafficking, dense granule biogenesis, and pulmonary fibrotic remodeling. Mutations in HPS1 cause Hermansky-Pudlak syndrome type 1, an autosomal recessive disorder characterized by oculocutaneous albinism, prolonged bleeding, and progressive pulmonary fibrosis, with HPS-1 being the clinically most severe subtype and particularly prevalent in northwest Puerto Rico due to a founder duplication (1470_1486dup16).

Supplied as 100 µl of liquid antiserum and intended for research use only, the reagent is compatible with standard immunodetection protocols across fixed tissue sections, cultured cell lines, and lysate-based blots, making it a versatile tool for laboratories studying rare pigment disorders, platelet granule formation, and the cellular pathology of LRO dysfunction.