Molecular Genetic Epidemiology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany.
Mol Genet Genomics. 2022 Jul;297(4):965-979. doi: 10.1007/s00438-022-01896-0. Epub 2022 May 13.
About 15% of colorectal cancer (CRC) patients have first-degree relatives affected by the same malignancy. However, for most families the cause of familial aggregation of CRC is unknown. To identify novel high-to-moderate-penetrance germline variants underlying CRC susceptibility, we performed whole exome sequencing (WES) on four CRC cases and two unaffected members of a Polish family without any mutation in known CRC predisposition genes. After WES, we used our in-house developed Familial Cancer Variant Prioritization Pipeline and identified two novel variants in the solute carrier family 15 member 4 (SLC15A4) gene. The heterozygous missense variant, p. Y444C, was predicted to affect the phylogenetically conserved PTR2/POT domain and to have a deleterious effect on the function of the encoded peptide/histidine transporter. The other variant was located in the upstream region of the same gene (GRCh37.p13, 12_129308531_C_T; 43 bp upstream of transcription start site, ENST00000266771.5) and it was annotated to affect the promoter region of SLC15A4 as well as binding sites of 17 different transcription factors. Our findings of two distinct variants in the same gene may indicate a synergistic up-regulation of SLC15A4 as the underlying genetic cause and implicate this gene for the first time in genetic inheritance of familial CRC.
约 15%的结直肠癌(CRC)患者有一级亲属患有同样的恶性肿瘤。然而,对于大多数家庭来说,CRC 家族聚集的原因尚不清楚。为了确定 CRC 易感性的新型高至中度外显率种系变异,我们对一个没有已知 CRC 易感性基因突变的波兰家族的四个 CRC 病例和两个无影响的成员进行了全外显子测序(WES)。WES 后,我们使用我们内部开发的家族性癌症变异优先化管道,在溶质载体家族 15 成员 4(SLC15A4)基因中鉴定出两个新的变体。杂合错义变体 p. Y444C 预测会影响系统发育保守的 PTR2/POT 结构域,并对编码肽/组氨酸转运蛋白的功能产生有害影响。另一个变体位于同一基因的上游区域(GRCh37.p13,12_129308531_C_T;转录起始位点上游 43bp,ENST00000266771.5),并被注释为影响 SLC15A4 的启动子区域以及 17 种不同转录因子的结合位点。我们在同一个基因中发现了两个不同的变体,这可能表明 SLC15A4 的协同上调是潜在的遗传原因,并首次将该基因与家族性 CRC 的遗传有关。