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Name :
HPS4 antibody

Documents :
DataSheet Material Safety Data Sheets (MSDS)

Description :
HPS4 Rabbit Polyclonal antibody. Positive IHC detected in human liver tissue. Positive IF detected in HepG2 cells. Positive IP detected in Hela cells. Positive WB detected in A375 cells, HeLa cells, K-562 cells. Observed molecular weight by Western-blot: 70 kDa and 90 kDa

Tested applications :

Species reactivity :
Human; other species not tested.

Alternative names :
bK1048E9.4 antibody; bK1048E9.5 antibody; Hermansky Pudlak syndrome 4 antibody; HPS4 antibody; KIAA1667 antibody; Light ear protein homolog antibody

Immunogen :

Isotype :
Rabbit IgG

Preparation :
This antibody was obtained by immunization of HPS4 recombinant protein (Accession Number: XM_017029044). Purification method: Antigen affinity purified.

Clonality :

Formulation :
PBS with 0.1% sodium azide and 50% glycerol pH 7.3.

Storage instructions :
Store at -20℃. DO NOT ALIQUOT

Applications :
Recommended Dilution: WB: 1:200-1:1000IP: 1:200-1:2000IHC: 1:20-1:200IF: 1:10-1:100

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.

References :
Ikawa Y, Hess R, Dorward H. In vitro functional correction of Hermansky-Pudlak Syndrome type-1 by lentiviral-mediated gene transfer. Molecular genetics and metabolism. 114(1):62-5. 2015.

Antibodies are immunoglobulins secreted by effector lymphoid B cells into the bloodstream. Antibodies consist of two light peptide chains and two heavy peptide chains that are linked to each other by disulfide bonds to form a “Y” shaped structure. Both tips of the “Y” structure contain binding sites for a specific antigen. Antibodies are commonly used in medical research, pharmacological research, laboratory research, and health and epidemiological research. They play an important role in hot research areas such as targeted drug development, in vitro diagnostic assays, characterization of signaling pathways, detection of protein expression levels, and identification of candidate biomarkers.
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Author: Gardos- Channel