Sorbonne Université, Département de génétique, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, F-75013, Paris, France.
GenoDiag, Paris, France.
Eur J Hum Genet. 2022 Oct;30(10):1187-1190. doi: 10.1038/s41431-022-01064-3. Epub 2022 Mar 11.
Despite routine analysis of a large panel of genes, pathogenic variants are only detected in approximately 20% of families with hereditary breast and/or ovarian cancer. Mobile element insertions (MEI) are known to cause genetic diseases in humans, but remain challenging to detect. Retrospective analysis of targeted next-generation sequencing (NGS) data from 359 patients was performed using a dedicated MEI detection pipeline. We detected one MEI in exon 9 of the PALB2 gene in a woman with a family history of breast cancer. The pathogenic variant, c.2872_2888delins114AluL2, disrupts the PALB2 coding sequence and leads to the production of a truncated protein, p.(Gln958Valfs*38). This is the first report of a pathogenic MEI in PALB2. This study illustrates that MEI analysis may help to improve molecular diagnostic yield and can be performed from targeted NGS data used for routine diagnosis.
尽管对大量基因进行了常规分析,但在遗传性乳腺癌和/或卵巢癌的家族中,只有约 20%能检测到致病变体。移动元件插入(MEI)已知会导致人类遗传疾病,但仍难以检测。使用专门的 MEI 检测管道对 359 名患者的靶向下一代测序(NGS)数据进行了回顾性分析。我们在一名有乳腺癌家族史的女性的 PALB2 基因exon 9 中检测到一个 MEI。该致病变体 c.2872_2888delins114AluL2 破坏了 PALB2 编码序列,并导致截短蛋白的产生,p.(Gln958Valfs*38)。这是 PALB2 中致病性 MEI 的首次报道。本研究表明,MEI 分析可以帮助提高分子诊断的检出率,并且可以从用于常规诊断的靶向 NGS 数据中进行。