{"product_id":"yp-ab-03403-53ul","title":"HPS-1 Monoclonal Antibody","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eReaction species:\u003c\/b\u003e Human\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e HPS1\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein name:\u003c\/b\u003e Hermansky-Pudlak syndrome 1 protein\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e Purified recombinant fragment of HPS-1 expressed in E. Coli.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e HPS-1 Monoclonal Antibody detects endogenous levels of HPS-1 protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Monoclonal, Mouse\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e Western Blot: 1\/500 - 1\/2000. ELISA: 1\/10000. Not yet tested in other applications.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e Affinity purification\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eStorage:\u003c\/b\u003e -20°C\/1 year\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eOther Names:\u003c\/b\u003e HPS1; HPS; Hermansky-Pudlak syndrome 1 protein\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. The encoded protein is a component of three different protein complexes termed biogenesis of lysosome-related organelles complex (BLOC)-3, BLOC4, and BLOC5. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 1. Alternative splicing results in multiple transcript variants. A pseudogene related to this gene is located on chromosome 22. [provided by RefSeq, Aug 2015],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e alternative products:Additional isoforms seem to exist,disease:Defects in HPS1 are the cause of Hermansky-Pudlak syndrome type 1 (HPS1) [MIM:203300]. 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.,function:Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting.,online information:HPS1 mutations,online information:Retina International's Scientific Newsletter,tissue\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"53UL","offer_id":50688882704664,"sku":"YP-Ab-03403-53UL","price":0.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/images_26e16913-956e-4cde-8927-417832a1fa35.png?v=1758556869","url":"https:\/\/danabiosci.com\/products\/yp-ab-03403-53ul","provider":"Dana Bioscience","version":"1.0","type":"link"}