Department of Human Genetics & Metabolic Diseases, Hadassah Hebrew University Medical Center, Jerusalem, 91120, Israel.
Fam Cancer. 2011 Mar;10(1):59-63. doi: 10.1007/s10689-010-9395-9.
Founder mutations in BRCA1/2 genes have been detected in several Jewish communities in Israel, including in Ashkenazi Jews and Jews who immigrated to Israel from Iraq, Yemen, Iran and Afghanistan. We analyzed DNA samples of patients of Sephardic origin (descendents of Jews from the Iberian Peninsula) with breast cancer (BC) and/or ovarian cancer (OC) and additional family history of these cancers. In this study we identified 2 mutations: p.A1708E in BRCA1 and c.67 + 1G > A (IVS2 + 1G > A) in BRCA2, each in 3 unrelated patients. The frequency of the two mutations was 26-31% among Sephardic high risk families and about 3% among the full cohort of 177 patients of this origin who were tested in our center. Based on haplotype analysis we concluded that these mutations are most probably founder mutations in Sephardic Jews. We recommend testing the two mutations in women of Sephardic origin who apply for BRCA testing because of personal and/or family history of BC and/or OC. Furthermore, we suggest adding them to the 5 mutations included in "The Jewish panel" of BRCA1/2 mutations that are being tested in Israel.
BRCA1/2 基因中的种系突变已在以色列的几个犹太社区中被检测到,包括在阿什肯纳兹犹太人以及从伊拉克、也门、伊朗和阿富汗移民到以色列的犹太人中。我们分析了具有乳腺癌(BC)和/或卵巢癌(OC)以及这些癌症家族史的塞法迪血统(源自伊比利亚半岛的犹太人后裔)患者的 DNA 样本。在这项研究中,我们鉴定出了 2 种突变:BRCA1 中的 p.A1708E 和 BRCA2 中的 c.67+1G>A(IVS2+1G>A),每个突变都存在于 3 个不相关的患者中。这两种突变在塞法迪高危家族中的频率为 26-31%,而在我们中心检测的 177 名该血统的患者的全队列中,其频率约为 3%。基于单体型分析,我们得出结论,这些突变很可能是塞法迪犹太人中的种系突变。我们建议对因个人和/或家族的 BC 和/或 OC 病史而申请 BRCA 检测的塞法迪血统女性进行这两种突变的检测。此外,我们建议将它们添加到以色列正在检测的 BRCA1/2 突变的“犹太面板”中包含的 5 种突变中。