Abstract
Complement component C1 is composed of three subcomponents, C1q, C1r and C1s (refs 1, 2). The problem of which cells produce C1 in vivo has been controversial, and many cell types3–9 have been reported to synthesize these molecules. Material antigenically similar to C1q was reported10 to be synthesized and secreted by human and rat fibroblast cell lines. Reid and Solmon11 subsequently showed that C1 haemolytic activity was secreted into the medium of cultured human fibroblast cell lines, and that the form of the C1q was unusual in that it had an apparently higher molecular weight than did C1q isolated from normal serum. As fibroblasts are an abundant cell type, this observation suggested that they might be a major contributor to serum C1q, secreting C1q precursor, pro-C1q. We describe here a genetic defect of serum C1q. Homozygotes for this defect do not produce functional plasma C1q, although they seem to produce normal fibroblast C1q. Heterozygotes produce both normal and defective serum C1q. This suggests that different genes code for the chains of C1q produced by fibroblasts and for those produced in serum.
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Skok, J., Solomon, E., Reid, K. et al. Distinct genes for fibroblast and serum C1q. Nature 292, 549–551 (1981). https://doi.org/10.1038/292549a0
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DOI: https://doi.org/10.1038/292549a0
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