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HPS6 Polyclonal Antibody

Purified Rabbit Polyclonal Antibody (Pab)

     
  • WB - HPS6 Polyclonal Antibody AP56681
    Sample:
    293T(Human) Cell Lysate at 30 ug
    Primary: Anti-HPS6 (bs-17384R) at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 83 kD
    Observed band size: 83 kD
    detail
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immuno electron microscopy
  • EIA=Enzyme Immunoassay
WB, IHC-P, IHC-F, IF, ICC, E
Primary Accession Q86YV9
Reactivity Rat, Pig, Bovine
Host Rabbit
Clonality Polyclonal
Calculated MW 83 KDa
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human HPS6
Epitope Specificity 501-600/775
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Microsome membrane. Cytoplasm > cytosol. Early endosome membrane.
DISEASE Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:614075]. 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This intronless gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. This protein interacts with Hermansky-Pudlak syndrome 5 protein. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 6. [provided by RefSeq, Jul 2008]
Additional Information
Gene ID 79803
Other Names Hermansky-Pudlak syndrome 6 protein, Ruby-eye protein homolog, Ru, HPS6
Target/Specificity Ubiquitous.
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,ELISA=1:5000-10000
Format0.01M TBS(pH7.4), 0.09% (W/V) sodium azide and 50% Glyce
StorageStore at -20 ℃ for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 ℃.
Protein Information
Name HPS6
Function May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules (PubMed:17041891). Acts as a cargo adapter for the dynein- dynactin motor complex to mediate the transport of lysosomes from the cell periphery to the perinuclear region. Facilitates retrograde lysosomal trafficking by linking the motor complex to lysosomes, and perinuclear positioning of lysosomes is crucial for the delivery of endocytic cargos to lysosomes, for lysosome maturation and functioning (PubMed:25189619).
Cellular Location Microsome membrane. Cytoplasm, cytosol. Early endosome membrane. Lysosome membrane
Tissue Location Ubiquitous.
Research Areas
Citations (0)
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$ 385.00
Cat# AP56681
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