The protein CD63 is in platelet dense granules, is deficient in a patient with Hermansky-Pudlak syndrome, and appears identical to granulophysin
| Autor: | Archibald McNicol, Masahiro Nishibori, Nidhi Jain, Abraham Shalev, Bonnie Cham, Jon M. Gerrard |
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| Rok vydání: | 1993 |
| Předmět: |
Adult
Blood Platelets Male Serotonin Blotting Western Molecular Sequence Data Enzyme-Linked Immunosorbent Assay Platelet Membrane Glycoproteins Cytoplasmic Granules Immunofluorescence Platelet membrane glycoprotein Antigen Antigens CD medicine Humans Platelet Amino Acid Sequence Staining and Labeling medicine.diagnostic_test CD63 biology Tetraspanin 30 General Medicine medicine.disease Immunohistochemistry Molecular biology Biochemistry Albinism Oculocutaneous biology.protein Hermansky–Pudlak syndrome Dense granule Antibody Lysosomes Research Article |
| Zdroj: | Journal of Clinical Investigation. 91:1775-1782 |
| ISSN: | 0021-9738 |
| DOI: | 10.1172/jci116388 |
| Popis: | The levels and expression of the proteins CD63 and granulophysin in platelets from control and from a Hermansky-Pudlak syndrome subject (a condition characterized by dense granule and lysosomal deficiencies and the accumulation of ceroid-like material in reticuloendothelial cells) were examined. Immunofluorescence studies indicated that anti-CD63 and anti-granulophysin antibodies recognized similar numbers of granules; coapplication of antibodies did not identify more granules than the individual antibodies. Significantly fewer granules were recognized in Hermansky-Pudlak syndrome platelets than in control using either antibody. Immunoblotting studies demonstrated that anti-CD63 and anti-granulophysin antibodies apparently recognize the same protein, which was deficient in Hermansky-Pudlak platelets. Analysis by fluorescence-activated cell sorter (FACS) showed biphasic expression of CD63 and granulophysin after thrombin stimulation of control but not Hermansky-Pudlak platelets. Anti-CD63 effectively blocked detection of the protein by anti-granulophysin using immunofluorescence, ELISA, immunoblotting, and FACS analysis. Amino-terminal sequencing over the first 37 amino acids revealed that granulophysin was homologous to CD63, melanoma antigen ME491, and pltgp40. These results suggest that granulophysin and CD63 are possibly identical proteins. This is the first report of a protein present in platelet dense granules, lysosomes, and melanocytes, but deficient in a patient with Hermansky-Pudlak syndrome. |
| Databáze: | OpenAIRE |
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