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Anti-HPS4 Antibody

Boster Bio Anti-HPS4 Antibody catalog # A06241-1. Tested in WB, IHC, ICC, IF, IP, Flow Cytometry, ELISA applications. This antibody reacts with Human.

Product Specifications

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.

Synonyms

Light-ear protein homolog; KIAA1667; Hermansky-Pudlak syndrome 4 protein; BLOC-3 complex member HPS4

Gene Name

HPS4

UniProt

Q9NQG7

Host

Rabbit

Reactivity

Human

Immunogen

E.coli-derived human HPS4 recombinant protein (Position: T3-E348) .

Clonality

Polyclonal

Applications

WB, IHC, ICC, IF, IP, Flow Cytometry, ELISA

Purification

Immunogen affinity purified.

Concentration

500 μg/mL

Form

Liquid

Storage Conditions

12 months from date of receipt, -20°C as supplied. 6 months 2 to 8°C after reconstitution. Avoid repeated freezing and thawing.

Gene Name Synonym

HPS4 biogenesis of lysosomal organelles complex 3 subunit 2

Protein Name

BLOC-3 complex member HPS4

Contents

500 μg/mL antibody with PBS,0.02% NaN3, 1 mg BSA and 50% glycerol.

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