HPS4 Antibody 2 Publications

Rabbit Polyclonal| Catalog number: 14627-1-AP

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Con: 20 μg/150 μl

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Species specificity:
human

Positive WB detected in:
A375 cells, HeLa cells, K-562 cells

Positive IP detected in:
Hela cells

Positive IHC detected in:
human liver tissue

Positive IF detected in:
HepG2 cells

Recommended dilution:
WB : 1:200-1:1000
IP : 0.5-4.0 ug for IP and 1:500-1:1000 for WB
IHC : 1:20-1:200
IF : 1:10-1:100

Product Information


Source:
Rabbit

Purification method:
Antigen affinity purification

Isotype:
IgG

Storage:
PBS with 0.1% sodium azide and 50% glycerol pH 7.3. Store at -20oC. Aliquoting is unnecessary for -20oC storage.

Immunogen Information


Full name:
Hermansky-Pudlak syndrome 4

Calculated molecular weight:
77 kDa

Observed molecular weight:
70 kDa and 90 kDa

GenBank accession number:

Gene ID (NCBI):

Gene symbol
HPS4

Synonyms
bK1048E9.4, bK1048E9.5, Hermansky Pudlak syndrome 4, HPS4, KIAA1667, LE, Light ear protein homolog
Background

Hermansky-Pudlak syndrome (HPS) is a genetic disease 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. HPS1 and HPS4 are the most frequently mutated genes associated with HPS in humans. Both of HPS1 and HPS4 are components of two complexes involved in biogenesis of melanosome and lysosome-related organelles: BLOC-3 and BLOC-4. HPS4 is supposed to interact with HPS1 and stabilize HPS1. The human HPS4 migrates at about 90 kDa on SDS-PAGE, versus its predicated molecular mass of 77 kDa.


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