Priority Research Centre for Reproductive Science, College of Engineering, Science and Environment, The University of Newcastle, Callaghan New South Wales, Australia; Hunter Medical Research Institute, New Lambton Heights New South Wales, Australia.
Reprod Biomed Online. 2022 Jul;45(1):109-124. doi: 10.1016/j.rbmo.2022.03.012. Epub 2022 Mar 17.
Spermatogonial stem cells exhibit a low level of spontaneous mutation that is heavily impacted by paternal age via mechanisms that appear to involve the aberrant repair of DNA damage. This background de-novo mutation frequency can be increased 1000-fold by mutations affecting a key signal transduction pathway that confers upon its descendants a selective advantage, leading to clonal expansion and nests of mutant germ cells in the testes of ageing males. This 'selfish selection' model effectively explains the origin of several dominant developmental disorders, such as achondroplasia and Apert syndrome, but cannot be generalized to account for the majority of de-novo mutations where no selective advantage is apparent. In this article, an additional germline mutation pathway is proposed that recognizes the unique susceptibility of spermatozoa to DNA damage and the importance of the oocyte in repairing these lesions prior to the S phase of the first mitotic division. Any deficiency or inaccuracy on the part of the oocyte in effecting this repair process has the potential to fix paternal DNA damage as a de-novo mutation in the embryo. Such a mechanism supports emerging data indicating that assisted conception procedures may enhance the mutational load carried by ART offspring.
精原干细胞自发突变率较低,但会受到父系年龄的严重影响,其机制似乎涉及 DNA 损伤的异常修复。通过影响关键信号转导通路的突变,这种背景下的新生突变频率可增加 1000 倍,该通路赋予其后代选择优势,导致睾丸中衰老雄性的精原细胞克隆扩张和突变巢。这种“自私选择”模型有效地解释了几种显性发育障碍的起源,如软骨发育不全症和 Apert 综合征,但不能推广到大多数新生突变,因为这些突变没有明显的选择优势。在本文中,提出了另一种种系突变途径,该途径认识到精子对 DNA 损伤的独特易感性,以及卵母细胞在第一次有丝分裂分裂 S 期之前修复这些损伤的重要性。卵母细胞在进行这种修复过程中任何缺陷或不准确都有可能将父系 DNA 损伤固定为胚胎中的新生突变。这种机制支持新兴数据表明,辅助受孕程序可能会增加 ART 后代携带的突变负荷。