| CatalogCode: | NB100-1445 |
| ProductName: | HPS3 Antibody |
| Product Description: | Goat Polyclonal anti-HPS3 |
| Clonality: | Polyclonal |
| Immunogen: | Synthetic peptide: PYLLYCSRKKPLT, representing the C-terminus of the human protein (residues 992-1004), according to NP_115759. |
| CrossReactivity: | Tested: Human. Expected to cross-react with Human due to sequence homology. Not yet tested in other species. |
| Packaging: | 0.1 mg Immunogen affinity purified Goat antisera. |
| Uses: | This antibody can be used for ELISA and Western Blot. In Western Blot, detects a band of approximately 113 kDa (predicted molecular weight: 113 kDa). Not tested in other applications.Recommended Starting Dilution:*ELISA: 1/32000WB: 1 - 3 ug/ml*Optimal dilutions/concentrations should be determined by the end user. |
| Localization: | Cytoplasmic |
| Background: | HPS3 is involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa) mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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. |
| Storage: | Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze thaw cycles. |
| Purity: | Immunogen affinity purified |
| Isotype: | IgG |
| Host_Name: | Goat |
| Buffer: | Tris saline containing [pH7.3] with 0.5% BSA |
| ListPrice: | 295 |
| AppSummary: | ELISA, WB |
| SpeciesSummary: | Hu |
| ALTnames: | anti-Cocoa antibody, anti-FLJ22704 antibody, anti-Hermansky Pudlak syndrome 3 protein antibody, anti-SUTAL antibody |
| ProteinTarget: | HPS3 |
| PackageSize: | 0.1 mg |
| GeneralRef: | Gautam R et al. The Hermansky-Pudlak syndrome 3 (cocoa) protein is a component of the biogenesis of lysosome-related organelles complex-2 (BLOC-2). J Biol Chem 279:12935-42 (2004). |
| Gene_Id: | 84343 |
| Omim_ID: | NB300-931 |
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