Effects of C3 Nephritic Factor on the Complement System

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  • Nakanishi Isao
    The First Department of Medicine, Osaka University Medical School, Osaka

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  • Ｃ３　ｎｅｐｈｒｉｔｉｃ　ｆａｃｔｏｒの補体系に及ぼす影響　　とくにＣ３ｂＢｂに対する安定化作用の検討
  • C3 nephrtic factor ノ ホタイケイ ニ オヨボス エイキョウ
  • ―とくにC3bBb対する安定化作用の検討―

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In the sera of three patients with partial lipodystropyh (PLD), CH50 and C3 hemolytic activity were markedly reduced, while the other complement components activities, especially C5 activity, were within normal limits. These sera showed high C3 nephritic factor (NeF) activities. The purpose of this paper is to clarify the effect of NeF on the complemet system focusing the attention to the stabilizing effect of NeF on C3 convertase (C3bBb) activity. NeF was partially purified from three PLD sera and each was active as a stabilizer for the alternative pathway C3 convertase (C3bBb), but not active for the classical pathway C3 convertase (C42). Though NeF was equally active as a stabilizer of C3bBb on EA as properdin (P), P stabilized C3bBb was easily decayed by the action of 131H globulin (β1H), while NeF stabilized C3bBb was resistant to β1HH action. Moreover, C3- and C5-cleaving activities on ZX formed in the serum with NeF was stable, while those activities on ZX formed in the serum without NeF decayed rapidly. NeF could stabilize C3 convertase in the fluid phase phase as well as on solid phase. The reaction mixture of purified proteins, P, B, D, C3b-INA, β1H, C3, C5 and NeF, caused remarkable reduction of C3 activity, but C5 activity was not redused presumably due to the inefficient formation of C5 convertase (C3bnBb) in the fluid phase. This may be the cause of reduced C3 and normal C5 activities in the PLD serum.

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