Inherited deficiency of the seventh component of complement: studies of C7-consuming activity

Acta Paediatrica Japonica; Overseas Edition
Y KoitabashiA Baba

Abstract

Neither the hemolytic activity nor the protein level of the seventh component of serum complement (C7) was detectable in an 8-year-old girl with nephritis, but in her parents and her brother, they were about half of the normal level. The patient was a homozygote type with a complete deficiency of C7 while her parents and brother were all heterozygote type with a partial deficiency of C7. C7-consuming activity was demonstrated in the native serum of the patient with complete C7 deficiency, and it was found that large amounts of C56 were readily generated upon incubation of the patient's serum with zymosan. It is proposed that the C7-consuming activity in the native serum of this patient is due to small amounts of C56 generated during the activation of serum complement by some kind of infection such as a common cold.

References

Nov 1, 1977·The Journal of Clinical Investigation·J M DelâgeE Prochazka
Oct 1, 1975·The Journal of Clinical Investigation·J T BoyerT S Zimmerman
Jul 1, 1973·The Journal of Clinical Investigation·N K DayR A Good
Jan 1, 1971·The Journal of Pediatrics·R J PickeringH Gewurz
Sep 1, 1981·Annals of Internal Medicine·H NishinoM Mushimoto

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Citations

Sep 9, 2006·Journal of the Formosan Medical Association = Taiwan Yi Zhi·Yi-Chi ChiangPei-Hsuan Liang

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