Hermansky-Pudlak Syndrome 1 (HPS1) antibody

Details for Product No. ABIN969198
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Antigen
Synonyms HPS1, HPS, MGC123240, im:7143944, zgc:123240
Reactivity
Human
(12), (2), (2)
Host
Mouse
(10), (2)
Clonality (Clone)
Monoclonal ()
Application
ELISA, Western Blotting (WB)
(12), (9)
Pubmed 3 references available
Quantity 100 μL
Options
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Catalog No. ABIN969198
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Immunogen Purified recombinant fragment of HPS1 expressed in E. coli.
Clone 5G12G2
Isotype IgG2a
Purification Ascites
Alternative Name HPS1
Background Synonyms:
HPS, MGC5277, HPS1
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.
Gene ID 3257
Research Area Signaling
Application Notes Recommended Dilution:
ELISA: 1/10000, WB: 1/500 - 1/2000
Not yet tested in other applications.
Determining optimal working dilutions by titration test.
Restrictions For Research Use only
Format Liquid
Buffer Ascitic fluid containing 0.03 % sodium azide.
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/-20 °C
Storage Comment Store at 4 °C or at -20 °C for long term.
Supplier Images
anti-Hermansky-Pudlak Syndrome 1 (HPS1) antibody Figure 1: Western blot analysis using HPS1 mouse mAb against truncated HPS1 recombinant protein (1) and HPS1-hIgGFc transfected CHO-K1 cell lysate (2) .
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).

Fréchette, Leit, Woo et al.: "4-(Heteroarylaminomethyl)-N-(2-aminophenyl)-benzamides and their analogs as a novel class of histone deacetylase inhibitors." in: Bioorganic & medicinal chemistry letters, Vol. 18, Issue 4, pp. 1502-6, 2008 (PubMed).

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