The synthesis and secretion of factor H, a regulatory protein of the complement system, were
studied in skin fibroblasts from an H-deficient child who has chronic hypocomplementemic
renal disease. In normal fibroblasts, factor H transcripts of 4.3 and 1.8 kilobase pairs (kb)
encode a 155-kDa protein containing short consensus repeat (SCR) domains 1–20 and a
45-kDa protein which contains SCRs 1–7, respectively. The patient's fibroblasts expressed
normal amounts of the 4.3-and 1.8-kb messages constitutively and after tumor necrosis …