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Two distinct abnormalities in patients with C8 alpha-gamma deficiency. Low level of C8 beta chain and presence of dysfunctional C8 alpha-gamma subunit.
Tedesco, F; Roncelli, L; Petersen, B H; Agnello, V; Sodetz, J M.
Affiliation
  • Tedesco F; Istituto di Patologia Generale, Università di Trieste, Italy.
J Clin Invest ; 86(3): 884-8, 1990 Sep.
Article in En | MEDLINE | ID: mdl-2394837
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.
Subject(s)

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Complement C8 Limits: Humans Language: En Journal: J Clin Invest Year: 1990 Document type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Complement C8 Limits: Humans Language: En Journal: J Clin Invest Year: 1990 Document type: Article