HPS4 Polyclonal antibody

HPS4 Polyclonal Antibody for IF, IHC, IP, WB, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human and More (1)

Applications

WB, IP, IHC, IF, ELISA

Conjugate

Unconjugated

Cat no : 14627-1-AP

Synonyms

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



Tested Applications

Positive WB detected inA375 cells, HeLa cells, K-562 cells
Positive IP detected inHela cells
Positive IHC detected inhuman liver tissue
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
Positive IF detected inHepG2 cells

Recommended dilution

ApplicationDilution
Western Blot (WB)WB : 1:200-1:1000
Immunoprecipitation (IP)IP : 0.5-4.0 ug for IP and 1:500-1:1000 for WB
Immunohistochemistry (IHC)IHC : 1:20-1:200
Immunofluorescence (IF)IF : 1:10-1:100
Sample-dependent, check data in validation data gallery

Published Applications

WBSee 3 publications below

Product Information

14627-1-AP targets HPS4 in WB, IP, IHC, IF, ELISA applications and shows reactivity with human samples.

Tested Reactivity human
Cited Reactivity human, mouse
Host / Isotype Rabbit / IgG
Class Polyclonal
Type Antibody
Immunogen HPS4 fusion protein Ag6202
Full Name Hermansky-Pudlak syndrome 4
Calculated molecular weight 77 kDa
Observed molecular weight 70 kDa and 90 kDa
GenBank accession numberBC065030
Gene symbol HPS4
Gene ID (NCBI) 89781
Conjugate Unconjugated
Form Liquid
Purification Method Antigen affinity purification
Storage Buffer PBS with 0.1% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.

Background Information

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.

Protocols

Product Specific Protocols
WB protocol for HPS4 antibody 14627-1-APDownload protocol
IHC protocol for HPS4 antibody 14627-1-APDownload protocol
IF protocol for HPS4 antibody 14627-1-APDownload protocol
Standard Protocols
Click here to view our Standard Protocols

Publications

SpeciesApplicationTitle
mouseWB

Am J Respir Cell Mol Biol

Gene-edited MLE-15 Cells as a Model for the Hermansky Pudlak Syndromes.

Authors - Seunghyi Kook
humanWB

Mol Genet Metab

In vitro functional correction of Hermansky-Pudlak Syndrome type-1 by lentiviral-mediated gene transfer.

Authors - Yasuhiro Ikawa
humanWB

PLoS One

Cellular and molecular defects in a patient with Hermansky-Pudlak syndrome type 5.

Authors - Joshi Stephen