Delvig A A, Kukharenko V I, Belkin V M, Mazurov V I, Grinberg K N, Debov S S
Institute of Medical Enzymology AMS USSR, Moscow.
Mol Gen Genet. 1987 Oct;209(3):592-5. doi: 10.1007/BF00331168.
Collagen and fibronectin synthesis by trisomic and triploid fibroblasts derived from human spontaneous abortuses was studied. It was demonstrated that the level of fibronectin and collagen production in fibroblasts with trisomy 7, trisomy 9, and triploidy was reduced as compared with diploid cells. A correlation between this observation and an increased rate of intracellular 14C-procollagen degradation was also established for the anomalous strains. No difference in hydroxylation of 14C-proline residues in alpha 1(I) and alpha 2(I) collagen chains and no fluctuation in the collagen type (I): type III ratio was found in the strains with the abnormal karyotypes. It was concluded that differentiation of the abnormal fibroblasts was impaired. The data also favour the hypothesis that the deficiency of the fibroblasts in producing proteins may account for a variety of anatomic abnormalities of embryos.
对源自人类自然流产的三体和三倍体成纤维细胞的胶原蛋白和纤连蛋白合成进行了研究。结果表明,与二倍体细胞相比,7号三体、9号三体和三倍体成纤维细胞中纤连蛋白和胶原蛋白的产生水平降低。对于异常菌株,还确定了这一观察结果与细胞内14C-前胶原降解速率增加之间的相关性。在核型异常的菌株中,未发现α1(I)和α2(I)胶原链中14C-脯氨酸残基的羟化存在差异,且I型胶原与III型胶原的比例也无波动。得出的结论是,异常成纤维细胞的分化受到损害。这些数据也支持这样一种假说,即成纤维细胞产生蛋白质的缺陷可能是胚胎各种解剖学异常的原因。