Rabbit HPS1 Polyclonal Antibody | anti-HPS1 antibody

HPS1 Polyclonal Antibody

Cat. Num. AAA3011629

• Gene Names: HPS1; HPS; BLOC3S1
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: HPS1; Polyclonal Antibody; HPS1 Polyclonal Antibody; Hermansky-Pudlak syndrome 1 protein; HPS; anti-HPS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: HPS1 polyclonal antibody detects endogenous levels of HPS1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 700

• Applicable Applications for anti-HPS1 antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: WB: 1:500-1:2000; IHC: 1:50-1:200; IF: 1:50-1:200
• Immunogen: Recombinant full length Human HPS1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded mouse stomach using HPS1 antibody at dilution of 1:100 (x40 lens).)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded rat kidney using HPS1 antibody at dilution of 1:100 (x40 lens).)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human stomach using HPS1 antibody at dilution of 1:100 (x40 lens).)

Related Product Information for anti-HPS1 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.

NCBI and Uniprot Product Information

• NCBI GI #: 33286416
• NCBI GeneID: 3257
• NCBI Accession #: NP_000186.2
• NCBI GenBank Nucleotide #: NP_000186.2
• UniProt Accession #: Q92902
• Molecular Weight: ~ 79kDa
• NCBI Official Full Name: Hermansky-Pudlak syndrome 1 protein isoform a
• NCBI Official Synonym Full Names: HPS1 biogenesis of lysosomal organelles complex 3 subunit 1
• NCBI Official Symbol: HPS1
• NCBI Official Synonym Symbols: HPS; BLOC3S1
• NCBI Protein Information: Hermansky-Pudlak syndrome 1 protein
• UniProt Protein Name: Hermansky-Pudlak syndrome 1 protein
• Protein Family: Hermansky-Pudlak syndrome 1 protein
• UniProt Gene Name: HPS1
• UniProt Synonym Gene Names: HPS
• UniProt Entry Name: HPS1_HUMAN

NCBI Description

This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. The encoded protein is a component of three different protein complexes termed biogenesis of lysosome-related organelles complex (BLOC)-3, BLOC4, and BLOC5. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 1. Alternative splicing results in multiple transcript variants. A pseudogene related to this gene is located on chromosome 22. [provided by RefSeq, Aug 2015]