製品の概要

製品の詳細

  • 由来
    Synthetic
  • アミノ酸配列
    • 配列
      PYLLYCSRKKPLT
    • 領域
      992 to 1004

関連製品

特性

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.

  • アプリケーション

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

  • 製品の状態
    Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

関連情報

  • 別名
    • 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
  • 関連性
    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.
  • 細胞内局在
    Cytoplasmic

参考文献

ab45598 has not yet been referenced specifically in any publications.

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

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