Reid K B, Bentley D R, Wood K J
Philos Trans R Soc Lond B Biol Sci. 1984 Sep 6;306(1129):345-54. doi: 10.1098/rstb.1984.0095.
Normal human C1q is a serum glycoprotein of 460 kDa containing 18 polypeptide chains (6A, 6B, 6C) each 226 amino acids long and each containing an N-terminal collagen-like domain and a C-terminal globular domain. Two unusual forms of C1q have been described: a genetically defective form, which has a molecular mass of approximately 160 kDa and is found in the sera of homozygotes for the defect who show a marked susceptibility to immune complex related disease; a fibroblast form, shown to be synthesized and secreted, in vitro, with a molecular mass of about 800 kDa and with chains approximately 16 kDa greater than those of normal C1q. A higher than normal molecular mass form of C1q has also been described in human colostrum and a form of C1q has been claimed to represent one of the types of Fc receptor on guinea-pig macrophages. To initiate studies, at the genomic level, on these various forms of C1q, and to investigate the possible relation between the C1q genes and the procollagen genes, the complementary DNA corresponding to the B chain of normal C1q has been cloned and characterized.
正常人C1q是一种460 kDa的血清糖蛋白,包含18条多肽链(6A、6B、6C),每条链长226个氨基酸,每条链都含有一个N端胶原样结构域和一个C端球状结构域。已描述了两种异常形式的C1q:一种是基因缺陷形式,分子量约为160 kDa,在该缺陷的纯合子血清中发现,这些纯合子对免疫复合物相关疾病表现出明显的易感性;一种是成纤维细胞形式,体外实验表明其由成纤维细胞合成并分泌,分子量约为800 kDa,其链比正常C1q的链大约大16 kDa。在人初乳中也描述了一种分子量高于正常的C1q形式,并且有人声称一种C1q形式代表豚鼠巨噬细胞上的一种Fc受体类型。为了在基因组水平上对这些各种形式的C1q展开研究,并探究C1q基因与前胶原基因之间的可能关系,已克隆并鉴定了与正常C1q的B链对应的互补DNA。