Mercier B, Lissens W, Audrézet M P, Bonduelle M, Liebaers I, Ferec C
Centre de Biogénétique, Brest, France.
Hum Mutat. 1993;2(1):16-20. doi: 10.1002/humu.1380020104.
We have analysed 194 Belgian CF chromosomes using a variety of techniques: delta F508 was detected by polyacrylamide gel electrophoresis; dot blotting of PCR products was used to identify the mutations G542X, 1717-1 G-->A, and N1303K; molecular defects in exons 2, 3, 4, 5, 6b, 7, 11, 12, 13, 14a, 14b, 17b, 19, 20, and 21 were screened for by DGGE. We identified 17 mutations, which accounted for 94.3% of the Belgian CF chromosomes. Four novel mutations and a novel polymorphism were characterized. The detection of such a high proportion of Belgian CF mutations is important in understanding the functional role of the molecule and in improving prenatal and genetic diagnosis of CF.
我们运用多种技术分析了194条比利时囊性纤维化(CF)染色体:通过聚丙烯酰胺凝胶电泳检测ΔF508;采用PCR产物斑点杂交法鉴定G542X、1717 - 1 G→A和N1303K突变;通过变性梯度凝胶电泳(DGGE)筛查外显子2、3、4、5、6b、7、11、12、13、14a、14b、17b、19、20和21中的分子缺陷。我们鉴定出17种突变,这些突变占比利时CF染色体的94.3%。对4种新突变和1种新多态性进行了特征描述。检测出如此高比例的比利时CF突变对于理解该分子的功能作用以及改善CF的产前和基因诊断具有重要意义。