Two distinct abnormalities in patients with C8 alpha-gamma deficiency. Low level of C8 beta chain and presence of dysfunctional C8 alpha-gamma subunit
| Autor: | Francesco Tedesco, J M Sodetz, L Roncelli, V Agnello, B H Petersen |
|---|---|
| Rok vydání: | 1990 |
| Předmět: |
Antiserum
medicine.diagnostic_test biology medicine.drug_class Macromolecular Substances Blotting Western Alpha (ethology) Enzyme-Linked Immunosorbent Assay General Medicine Monoclonal antibody Molecular biology Complement C8 Hemolysis Blot Western blot Polyclonal antibodies medicine biology.protein Humans Beta (finance) Gamma subunit Research Article |
| Zdroj: | The Journal of clinical investigation. 86(3) |
| ISSN: | 0021-9738 |
| Popis: | The sera from three C8 alpha-gamma deficient patients previously reported to have a selective C8 alpha-gamma defect were analyzed by SDS-PAGE and Western blot using two polyclonal antisera to C8 alpha-gamma and a monoclonal antibody to C8 alpha. All three sera exhibited C8 alpha-gamma bands that dissociated into alpha and gamma chains under reducing conditions. Quantitation of the alpha-gamma subunit in these sera by a sensitive ELISA revealed an amount approximately 1% of that found in normal human serum. A similar assay performed with a specific antiserum to C8 beta showed unexpectedly low levels of C8 beta in these sera, which were confirmed by hemolytic titration of C8 beta. The remarkable differences between C8 alpha-gamma and C8 beta in the C8 alpha-gamma deficient sera was that in spite of their comparable immunochemical levels, C8 beta still exhibited functional activity whereas C8 alpha-gamma was totally inactive. That the residual C8 alpha-gamma was inactive was also proved by its inability to show lytic bands in an overlay system after SDS-PAGE and subsequent removal of SDS. The implications of these findings for a novel concept of C8 deficiency are discussed. |
| Databáze: | OpenAIRE |
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