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HPS1 Rabbit anti-Human, Polyclonal, Proteintech
SDP

Catalog No. p-7206697 Shop All Proteintech Group Inc Products
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50-172-9957 150 μL
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Catalog No. 50-172-9957 Supplier Proteintech Group Inc Supplier No. 150771AP150UL
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Rabbit Polyclonal Antibody

Hermansky-Pudlak syndrome (HPS) is a rare, genetically heterogeneous, autosomal recessive disorder. It is characterized by oculocutaneous albinism, lysosomal storage defects and prolonged bleeding due to platelet storage pool deficiency. There are 10 HPS genes encoding HPS proteins that all interact within three distinct ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in these genes cause HPS. HSP-1 is a component of multiple cytoplasmic organelles and is important for their normal development and function. It plays a role in intracellular protein sorting.

Specifications

Antigen HPS1
Applications Immunocytochemistry, Immunofluorescence, Western Blot
Classification Polyclonal
Concentration 0.24 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene HPS1
Gene Accession No. Q92902
Gene Alias Hermansky Pudlak syndrome 1, HPS, HPS1
Gene Symbols HPS1
Host Species Rabbit
Immunogen HPS1 Fusion Protein Ag7149
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 3257
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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