HPS1
Reactivity: Human
Host: Rabbit
Polyclonal
Alexa Fluor 750
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
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).
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