Invitrogen™ HPS1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA563938

Catalog No. PIPA563938

Description

Immunogen sequence: LWQGINLVLL TRSPSAPLAL VLSQLMDGFS MLEKKLKEGP EPGASLRSQP LVGDLRQRMD KFVKNRGAQE IQSTWLEFKA KAFSK Highest antigen sequence identity to the following orthologs: Mouse - 75%, Rat - 74%.

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
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: HPS1
• Gene Alias: 6030422N11Rik; BB405864; BLOC3S1; ep; Gm21361; Hermansky-Pudlak syndrome 1 homolog (human); Hermansky-Pudlak syndrome 1 protein; Hermansky-Pudlak syndrome 1 protein homolog; Hermansky-Pudlak syndrome protein homolog; HPS; HPS1; HPS1, biogenesis of lysosomal organelles complex 3 subunit 1; MGC5277; pale ear
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3257
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin)
• Concentration: 0.4 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: Q92902
• Gene Symbols: HPS1
• Immunogen: Recombinant protein corresponding to Human HPS1. Recombinant protein control fragment (Product #RP-101171).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG