β Chain deficiency in three patients with dysfunctional C8 molecules

摘要: Structural and functional studies were performed on a dysfunctional C8 molecule present in the serum of two siblings an unrelated individual. The these three sera exhibited pattern partial immunologic identity with normal but was devoid activity. immunoprecipitated from control antihuman antiserum analyzed by SDS-PAGE using highly purified human as reference. A selective absence band 62,000 mol. wt observed immunoprecipitates containing C8. Experiments α-γ γ subunits showed that hemolytic activity deficient could be reconstituted addition β chain not dimer. Binding to C567 excluded following observations: (1) EAC 1–7 treated then washed lysed after subunit C9; (2) abnormal molecules did interfere consumption soluble complex SC5b-7.