Teboul Raphaël, Grabias Michalina, Zucman-Rossi Jessica, Letouzé Eric
Centre de Recherche des Cordeliers, Sorbonne Université, Université de Paris, INSERM, Paris, France.
Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
NAR Cancer. 2023 Mar 15;5(2):zcad014. doi: 10.1093/narcan/zcad014. eCollection 2023 Jun.
Somatic mutations can disrupt splicing regulatory elements and have dramatic effects on cancer genes, yet the functional consequences of mutations located in extended splice regions is difficult to predict. Here, we use a deep neural network (SpliceAI) to characterize the landscape of splice-altering mutations in cancer. In our in-house series of 401 liver cancers, SpliceAI uncovers 1244 cryptic splice mutations, located outside essential splice sites, that validate at a high rate (66%) in matched RNA-seq data. We then extend the analysis to a large pan-cancer cohort of 17 714 tumors, revealing >100 000 cryptic splice mutations. Taking into account these mutations increases the power of driver gene discovery, revealing 126 new candidate driver genes. It also reveals new driver mutations in known cancer genes, doubling the frequency of splice alterations in tumor suppressor genes. Mutational signature analysis suggests mutational processes that could give rise preferentially to splice mutations in each cancer type, with an enrichment of signatures related to clock-like processes and DNA repair deficiency. Altogether, this work sheds light on the causes and impact of cryptic splice mutations in cancer, and highlights the power of deep learning approaches to better annotate the functional consequences of mutations in oncology.
体细胞突变可破坏剪接调控元件,并对癌症基因产生显著影响,然而位于扩展剪接区域的突变的功能后果却难以预测。在此,我们使用深度神经网络(SpliceAI)来描绘癌症中剪接改变突变的全貌。在我们内部的401例肝癌系列中,SpliceAI发现了1244个隐匿性剪接突变,这些突变位于关键剪接位点之外,在匹配的RNA测序数据中具有很高的验证率(66%)。然后,我们将分析扩展到一个包含17714个肿瘤的大型泛癌队列,发现了超过100000个隐匿性剪接突变。考虑到这些突变可提高驱动基因发现的能力,揭示了126个新的候选驱动基因。它还揭示了已知癌症基因中的新驱动突变,使肿瘤抑制基因中剪接改变的频率增加了一倍。突变特征分析表明了在每种癌症类型中可能优先导致剪接突变的突变过程,其中与时钟样过程和DNA修复缺陷相关的特征富集。总之,这项工作揭示了癌症中隐匿性剪接突变的原因和影响,并突出了深度学习方法在更好注释肿瘤学中突变功能后果方面的作用。