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Details for Product No. ABIN966298

Hermansky-Pudlak Syndrome 1 (HPS1) antibody

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Antigen
Synonyms HPS, HPS1, MGC123240, im:7143944, zgc:123240
Reactivity
»Alternatives Human
Host
»Alternatives Mouse
Clonality (Clone) Monoclonal ()
Application
»Alternatives Western Blotting (WB), ELISA
Pubmed 3 references available
Catalog no. ABIN966298
Quantity 0.1 mL
Price
481.25 $   Plus shipping costs $45.00
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Availability Will be delivered in 5 to 7 Business Days
Immunogen Purified recombinant fragment of HPS1 expressed in E. Coli.
Clone 5G12G2
Isotype IgG2a
Purification Ascitic fluid
Alternative Name HPS1
Background Hermansky-Pudlak syndrome 1. This protein 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. Multiple transcript variants encoding distinct isoforms have been identified for this gene, the full-length sequences of some of these have not been determined yet.
Synonyms: HPS, MGC5277, HPS1
Gene ID 3257
Research Area Signaling
Application Notes Western Bloting: 1/500 - 1/2000.
ELISA: Propose dilution 1/10000.
Not yet tested in other applications.
Determining optimal working dilutions by titration test.
Restrictions For Research Use only
Preservative Sodium azide
Storage 4 °C
Product cited in: Nazarian, Falcón-Pérez, DellAngelica: "Biogenesis of lysosome-related organelles complex 3 (BLOC-3): a complex containing the Hermansky-Pudlak syndrome (HPS) proteins HPS1 and HPS4." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 100, Issue 15, pp. 8770-5, 2003 (PubMed).

Kobashi, Yoshida, Miyashita et al.: "Hermansky-Pudlak syndrome with interstitial pneumonia without mutation of HSP1 gene." in: Internal medicine (Tokyo, Japan), Vol. 44, Issue 6, pp. 616-21, 2005 (PubMed).

Huizing, Parkes, Helip-Wooley et al.: "Platelet alpha granules in BLOC-2 and BLOC-3 subtypes of Hermansky-Pudlak syndrome." in: Platelets, Vol. 18, Issue 2, pp. 150-7, 2007 (PubMed).

Alternatives for antigen "Hermansky-Pudlak Syndrome 1 (HPS1)", type "Antibodies"
Hosts (9), (2)
Reactivities (11), (2), (2)
Applications (11), (9)
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