Schrijver Iris, Külm Maigi, Gardner Phyllis I, Pergament Eugene P, Fiddler Morris B
Department of Pathology, L235, Stanford University Medical Center, 300 Pasteur Dr., Stanford, CA 94305, USA.
J Mol Diagn. 2007 Apr;9(2):228-36. doi: 10.2353/jmoldx.2007.060100.
In the Ashkenazi Jewish population, serious and lethal genetic conditions occur with relatively high frequency. A single test that encompasses the majority of population-specific mutations is not currently available. For comprehensive carrier screening and molecular diagnostic purposes, we developed a population-specific and inclusive microarray. The arrayed primer extension genotyping microarray carries 59 sequence variant detection sites, of which 53 are detectable bi-directionally. These sites represent the most common variants in Tay-Sachs disease, Bloom syndrome, Canavan disease, Niemann-Pick A, familial dysautonomia, torsion dystonia, mucolipidosis type IV, Fanconi anemia, Gaucher disease, factor XI deficiency, glycogen storage disease type 1a, maple syrup urine disease, nonsyndromic sensorineural hearing loss, familial Mediterranean fever, and glycogen storage disease type III. Several mutations in the selected disorders that are not prevalent per se in the Ashkenazi Jewish populations, as well pseudodeficiency alleles, are also included in the array. The initial technical evaluation of this microarray demonstrates that it is comprehensive, robust, sensitive, specific, and easily modifiable. This cost-effective array is based on a diversely applied platform technology and is suitable for both carrier screening and disease detection in Ashkenazi and Sephardic Jewish populations.
在阿什肯纳兹犹太人群体中,严重和致命的遗传疾病以相对较高的频率发生。目前尚无一种涵盖大多数群体特异性突变的单一检测方法。为了进行全面的携带者筛查和分子诊断,我们开发了一种群体特异性且包容性强的微阵列。阵列引物延伸基因分型微阵列带有59个序列变异检测位点,其中53个可双向检测。这些位点代表了泰-萨克斯病、布卢姆综合征、卡纳万病、尼曼-匹克病A型、家族性自主神经功能障碍、扭转性肌张力障碍、IV型粘脂贮积症、范可尼贫血、戈谢病、因子XI缺乏症、1a型糖原贮积病、枫糖尿症、非综合征性感音神经性听力损失、家族性地中海热和III型糖原贮积病中最常见的变异。所选疾病中一些本身在阿什肯纳兹犹太人群体中并不普遍的突变以及假缺陷等位基因也包含在该阵列中。对这种微阵列的初步技术评估表明,它具有全面性、稳健性、敏感性、特异性且易于修改。这种具有成本效益的阵列基于一种应用广泛的平台技术,适用于阿什肯纳兹和西班牙裔犹太人群体的携带者筛查和疾病检测。