Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Species

      Human
    • Sequence

      GDHTESEGDLRRKLYVLKYLFEVHFGLVTVDGHLIRKELRPPDLAQRVQL WEHFQSLLWTYSRLREQEQCFAVEALERLIHPQLCELCIEALERHVIQAV NTSPE
    • Amino acids

      96 to 200
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158694 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • Hermansky Pudlak syndrome 1 protein
    • Hermansky Pudlak syndrome gene
    • Hermansky Pudlak syndrome type 1
    • Hermansky-Pudlak syndrome 1
    • Hermansky-Pudlak syndrome 1 protein
    • HPS
    • HPS1
    • HPS1_HUMAN
    • MGC5277
    see all
  • Function

    Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting.
  • Tissue specificity

    Ubiquitous.
  • Involvement in disease

    Defects in HPS1 are the cause of Hermansky-Pudlak syndrome type 1 (HPS1) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, 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.
  • Information by UniProt

Images

  • ab158694 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158694 has not yet been referenced specifically in any publications.

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