Sorbi S, Nacmias B, Mortilla M, Forleo P, Piacentini S, Amaducci L
Department of Neurological and Psychiatric Sciences, University of Florence, Italy.
Neurochem Int. 1994 Jul;25(1):81-4. doi: 10.1016/0197-0186(94)90057-4.
We screened 11 families from different regions of Italy by direct sequencing of exon 17 of the APP gene. Two unrelated families carried the APP717 mutation segregating with the disease. These two families originate from two Italian regions which are considered genetically separate. Published studies have demonstrated the presence of the APP717 Val-->Ile mutation in kindreds of British or Japanese origin with early onset familial Alzheimer's disease. These data suggest that the APP717 mutation is not confined to islander families which may share common founders. From the molecular genetic point of view we also did linkage analysis. Several families, in fact, have not shown a linkage with chromosome 21 and the resolution of this dilemma required investigation of those pedigrees both with additional markers from chromosome 21 and with markers from other chromosomes.
我们通过对APP基因第17外显子进行直接测序,对来自意大利不同地区的11个家族进行了筛查。两个无血缘关系的家族携带与疾病相关的APP717突变。这两个家族来自意大利的两个被认为在基因上相互独立的地区。已发表的研究表明,在英国或日本血统的早发性家族性阿尔茨海默病亲属中存在APP717 Val→Ile突变。这些数据表明,APP717突变并不局限于可能有共同奠基人的岛民家族。从分子遗传学角度,我们还进行了连锁分析。事实上,有几个家族并未显示出与21号染色体的连锁关系,而解决这一困境需要用来自21号染色体的其他标记以及来自其他染色体的标记对这些家系进行研究。