HPS1 antibody (Hermansky-Pudlak Syndrome 1)

Details for Product anti-HPS1 Antibody No. ABIN966298, Supplier: Log in to see
Antigen
  • HPS1
  • HPS
  • MGC123240
  • im:7143944
  • zgc:123240
  • Hermansky-Pudlak syndrome 1
  • HPS1
  • hps1
  • Hps1
Reactivity
Human
22
4
4
Host
Mouse
11
11
Clonality (Clone)
Monoclonal ()
Application
ELISA, Western Blotting (WB)
20
12
5
3
1
1
1
Options
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Immunogen Purified recombinant fragment of HPS1 expressed in E. Coli.
Clone 5G12G2
Isotype IgG2a
Purification Ascitic fluid
Alternative Name HPS1 (HPS1 Antibody Abstract)
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
Format Liquid
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C
Background publications Huizing, Parkes, Helip-Wooley, White, Gahl: "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).

Product cited in: Kobashi, Yoshida, Miyashita, Niki, Matsushima: "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).

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).