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Anti -HPS3 (Hermansky Pudlak Syndrome 3 Protein, Cocoa, FLJ22704, SUTAL)
Anti -HPS3 (Hermansky Pudlak Syndrome 3 Protein, Cocoa, FLJ22704, SUTAL)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Polyclonal |
Goat |
Affinity Purified |
E B |
|
| 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. | | | Catalog # | H7045 | | Cellular Localization | Cytoplasmic | | Applications | Suitable for use in ELISA and Western Blot. Other applications not tested. | | Recommended Dilution | ELISA: 1:32,000. | | Western Blot: 1-3ug/ml. Predicted molecular weight 113kD. Detects a band of ~ 113kD. | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | | | May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | | CAS Number | n/a | | Clone Type | Polyclonal | | Isotype | IgG | | Host | Goat | | Source | Human | | Concentration | ~0.5mg/ml | | Form | Supplied as a liquid in TBS, pH 7.2, 0.5% BSA. | | Purity | Purified by immunoaffinity chromatography. | | Immunogen | Synthetic peptide: PYLLYCSRKKPLT, representing the C-terminus of the human protein (residues 992-1004), according to NP_115759. | | Specificity | Species Crossreactivity | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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