Ottini Laura, Falchetti Mario, Saieva Calogero, De Marco Manola, Masala Giovanna, Zanna Ines, Paglierani Milena, Giannini Giuseppe, Gulino Alberto, Nesi Gabriella, Mariani Costantini Renato, Palli Domenico
Department of Experimental Medicine and Pathology, University La Sapienza, 00161 Rome, Italy.
Carcinogenesis. 2004 Dec;25(12):2337-43. doi: 10.1093/carcin/bgh257. Epub 2004 Aug 19.
Gastric carcinomas (GCs) with high-level microsatellite instability (MSI-H) are characterized by widespread mutations at coding and non-coding mononucleotide repeats. Deletions at coding mononucleotide tracts are predicted to cause frameshift mutations and alter normal protein functions. Mutations affecting non-coding mononucleotide repeats may lead to functional consequences if they occur in gene regulatory regions. To investigate whether mutations in non-coding polypyrimidine tracts within cancer-related genes may contribute to the phenotype of MSI-H GCs, we analysed the poly(T)11 tract constituting an accessory splicing signal within the intron 4 of the MRE11 gene. Mutations at the intronic MRE11 poly(T)11 were evaluated by PCR-based assay in 27 MSI-H, 22 MSI-low and 29 MSI-negative GCs derived from a well-characterized series of GCs identified in a high-risk area in Tuscany, Central Italy. Deletion of 2 and 1 bp at the MRE11poly(T)11 were identified in 33 and 48% MSI-H GCs, respectively. Biallelic mutations were frequently observed (77%) in GCs harbouring 2 bp deletions. The presence of MRE11poly(T)11 2 bp deletion was associated with a totally absent or strongly reduced MRE11 immunostaining (P < 0.001) and with a positive GC family history (P = 0.046). Immunoblotting assays confirmed the absence of MRE11 expression in GCs with a 2 bp deletion. The relatively high frequency of the MRE11poly(T)11 mutations, the occurrence of biallelic mutations and the evidence of loss of protein expression indicate MRE11 as novel mutational target in MSI-H GC. Overall, our results indicate that MSI-associated mutations occurring in non-coding repeats may affect protein expression in MSI-H GC.
具有高水平微卫星不稳定性(MSI-H)的胃癌(GC)的特征是编码和非编码单核苷酸重复序列广泛存在突变。编码单核苷酸序列的缺失预计会导致移码突变并改变正常蛋白质功能。如果影响非编码单核苷酸重复序列的突变发生在基因调控区域,可能会导致功能后果。为了研究癌症相关基因中非编码聚嘧啶序列的突变是否可能导致MSI-H GC的表型,我们分析了构成MRE11基因内含子4中辅助剪接信号的聚(T)11序列。通过基于PCR的检测方法,对来自意大利中部托斯卡纳一个高风险地区鉴定的一系列特征明确的GC中的27例MSI-H、22例MSI-low和29例MSI阴性GC中的内含子MRE11聚(T)11突变进行了评估。在33%和48%的MSI-H GC中分别鉴定出MRE11聚(T)11缺失2 bp和1 bp。在携带2 bp缺失的GC中经常观察到双等位基因突变(77%)。MRE11聚(T)11 2 bp缺失的存在与MRE11免疫染色完全缺失或强烈降低相关(P < 0.001),并与GC家族史阳性相关(P = 0.046)。免疫印迹分析证实了2 bp缺失的GC中不存在MRE11表达。MRE11聚(T)11突变的相对高频率、双等位基因突变的发生以及蛋白质表达缺失的证据表明MRE11是MSI-H GC中的新突变靶点。总体而言,我们的结果表明,非编码重复序列中发生的MSI相关突变可能会影响MSI-H GC中的蛋白质表达。