Name: HPS-1 Monoclonal Antibody
SKU: WM0238
Price: 268 USD
Availability: InStock

HPS-1 Monoclonal Antibody

Ucallm_WM0238-50

Price range: $268.00 through $328.00

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50μl

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SKU: WM0238-50

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Product details

Background:
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],

Specifications

Size	50μL/100μL
Target	HPS-1
Reactivity	Human
Applications	WB, ELISA
MW(Calculated)	79kD
MW(Observed)
Host Species	Mouse
Isotype
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:500-1:2000; ELISA 1:10000; Not yet tested in other applications.
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification	Affinity purification
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	10B1
Immunogen	Purified recombinant fragment of HPS-1 expressed in E. Coli.
Specificity	HPS-1 Monoclonal Antibody detects endogenous levels of HPS-1 protein.
Gene Name	HPS1
Protein Name	Hermansky-Pudlak syndrome 1 protein
Other Name	HPS1; / HPS; / Hermansky-Pudlak syndrome 1 protein
Species	Human
Gene ID	3257
Uniprot	Q92902,
Species.1	Mouse
Gene ID.1
Uniprot.1	O08983
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	cytoplasm,lysosome,integral component of plasma membrane,cytoplasmic, membrane-bounded vesicle,BLOC-3 complex,cytoplasmic vesicle,
Tissue Expression	Ubiquitous.
Signaling_pathway
Research Areas
Function	Alternative products:Additional isoforms seem to exist,Disease:Defects in HPS1 are the cause of Hermansky-Pudlak syndrome type 1 (HPS1) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder 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. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.,Function:Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting.,online information:HPS1 mutations,online information:Retina International's Scientific Newsletter,tissue specificity:Ubiquitous.,

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HPS-1 Monoclonal Antibody

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HPS-1 Monoclonal Antibody

Product details

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