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Research Article Free access | 10.1172/JCI114789

Two distinct abnormalities in patients with C8 alpha-gamma deficiency. Low level of C8 beta chain and presence of dysfunctional C8 alpha-gamma subunit.

F Tedesco, L Roncelli, B H Petersen, V Agnello, and J M Sodetz

Istituto di Patologia Generale, Università di Trieste, Italy.

Find articles by Tedesco, F. in: PubMed | Google Scholar

Istituto di Patologia Generale, Università di Trieste, Italy.

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Istituto di Patologia Generale, Università di Trieste, Italy.

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Istituto di Patologia Generale, Università di Trieste, Italy.

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Istituto di Patologia Generale, Università di Trieste, Italy.

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Published September 1, 1990 - More info

Published in Volume 86, Issue 3 on September 1, 1990
J Clin Invest. 1990;86(3):884–888. https://doi.org/10.1172/JCI114789.
© 1990 The American Society for Clinical Investigation
Published September 1, 1990 - Version history
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Abstract

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.

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