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波兰乳腺癌和乳腺癌-卵巢癌家族中 BRCA2 基因的新种系突变。

Novel germline mutations in BRCA2 gene among breast and breast-ovarian cancer families from Poland.

机构信息

Endocrinology Department, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, WK Roentgen 5, 02-781, Warsaw, Poland.

出版信息

Fam Cancer. 2010 Sep;9(3):267-74. doi: 10.1007/s10689-010-9338-5.

Abstract

Identification of mutations in the BRCA2 gene and estimation of their clinical consequences for women and men treated in the Maria Sklodowska-Curie Memorial Cancer Center Warsaw, Poland in the years 1998-2008. The probands (97 women and 8 men) had a family history of breast and ovarian cancer (median age 46). The presence of molecular changes was examined in DNA isolated from peripheral blood lymphocytes. Germline mutations in 27 exons of the BRCA2 gene were screened by 'touchdown' PCR amplification, DHPLC and sequencing. Missense mutations were classified by multiple-sequences alignments of orthologous BRCA2 protein sequences with T-Coffee software. 39 molecular changes (8 novel) were identified in the BRCA2 gene in 105 investigated patients. In 12 patients the following pathogenic mutations were identified: 5467insT, 6174delT, 6192delAT, 6675delTA, 8141del5, 9152delT, 9326insA, 9631delC, IVS23-2A > G and E394X. The presence of 10 missense type mutations was detected including the following: D1420O, T1915 M, N3124I. The determination of pathogenic status of molecular variants detected in BRCA2 gene, described in the BIC mutation database as 'UV' depends on many parameters. Important is the assessment of the evolutionary conservation of their protein sequences and studying of the frequency of molecular variants detected in breast cancer patients and in population. A high diversity was found of the pathogenic mutations detected in BRCA2 gene in the Polish population.

摘要

鉴定 BRCA2 基因突变并评估其对波兰华沙 Maria Sklodowska-Curie 纪念癌症中心 1998-2008 年期间治疗的女性和男性患者的临床影响。这些先证者(97 名女性和 8 名男性)有乳腺癌和卵巢癌家族史(中位年龄 46 岁)。从外周血淋巴细胞中提取 DNA,检测其是否存在分子变化。采用“降落”PCR 扩增、DHPLC 和测序法筛选 BRCA2 基因 27 个外显子中的种系突变。采用 T-Coffee 软件对同源 BRCA2 蛋白序列进行多序列比对,对错义突变进行分类。在 105 例研究患者中发现 BRCA2 基因中的 39 种分子变化(8 种为新突变)。在 12 例患者中发现以下致病性突变:5467insT、6174delT、6192delAT、6675delTA、8141del5、9152delT、9326insA、9631delC、IVS23-2A>G 和 E394X。检测到 10 种错义突变型,包括:D1420O、T1915 M、N3124I。BRCA2 基因中检测到的分子变异体的致病性状态的确定,取决于许多参数。重要的是评估其蛋白序列的进化保守性,并研究在乳腺癌患者和人群中检测到的分子变异体的频率。在波兰人群中发现 BRCA2 基因中检测到的致病性突变具有高度多样性。

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