Overview

Description

  • Nature
    Synthetic
  • Amino Acid Sequence
    • Sequence
      PYLLYCSRKKPLT
    • Amino acids
      992 to 1004

Associated products

Specifications

Our Abpromise guarantee covers the use of ab45598 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Blocking - Blocking peptide for Anti-HPS3 antibody (ab19047)

  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.

General Info

  • Alternative names
    • BLOC2S1
    • Cocoa
    • DKFZp686F0413
    • FLJ22704
    • Hermansky Pudlak syndrome 3
    • Hermansky Pudlak syndrome 3 protein
    • HPS3, biogenesis of lysosomal organelles complex 2 subunit 1
    • SUTAL
    see all
  • Relevance
    HPS3 is involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa) mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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.
  • Cellular localization
    Cytoplasmic

References

ab45598 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab45598.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up