Institute of Pediatrics, Children's Hospital of Fudan University, the Institutes of Biomedical Sciences, and the State Key Laboratory of Genetic Engineering, Fudan University, Shanghai, 200032, China.
J Assist Reprod Genet. 2021 May;38(5):993-1002. doi: 10.1007/s10815-021-02196-z. Epub 2021 Apr 24.
Successful human reproduction requires gamete maturation, fertilization, and early embryonic development. Human oocyte maturation includes nuclear and cytoplasmic maturation, and abnormalities in the process will lead to infertility and recurrent failure of IVF/ICSI attempts. In addition, the quality of oocytes/embryos in the clinic can only be determined by morphological markers, and there is currently a lack of molecular markers for determining oocyte quality. As the number of patients undergoing IVF/ICSI has increased, many patients have been identified with recurrent IVF/ICSI failure. However, the genetic basis behind this phenotype remains largely unknown. In recent years, a few mutant genes have been identified by us and others, which provide potential molecular markers for determining the quality of oocytes/embryos. In this review, we outline the genetic determinants of abnormalities in the processes of oocyte maturation, fertilization, and early embryonic development. Currently, 16 genes (PATL2, TUBB8, TRIP13, ZP1, ZP2, ZP3, PANX1, TLE6, WEE2, CDC20, BTG4, PADI6, NLRP2, NLRP5, KHDC3L, and REC114) have been reported to be the causes of oocyte maturation arrest, fertilization failure, embryonic arrest, and preimplantation embryonic lethality. These abnormalities mainly have Mendelian inheritance patterns, including both dominant inheritance and recessive inheritance, although in some cases de novo mutations have also appeared. In this review, we will introduce the effects of each gene in the specific processes of human early reproduction and will summarize all known variants in these genes and their corresponding phenotypes. Variants in some genes have specific effects on certain steps in the early human reproductive processes, while other variants result in a spectrum of phenotypes. These variants and genetic markers will lay the foundation for individualized genetic counseling and potential treatments for patients and will be the target for precision treatments in reproductive medicine.
人类生殖的成功需要配子成熟、受精和早期胚胎发育。人类卵母细胞成熟包括核和细胞质成熟,过程中的异常将导致不孕和反复体外受精/卵胞浆内单精子注射(IVF/ICSI)尝试失败。此外,临床中卵母细胞/胚胎的质量只能通过形态学标记来确定,目前缺乏用于确定卵母细胞质量的分子标记。随着接受 IVF/ICSI 的患者数量增加,许多患者被诊断为反复 IVF/ICSI 失败。然而,这种表型背后的遗传基础在很大程度上仍然未知。近年来,我们和其他人已经确定了一些突变基因,这些基因为确定卵母细胞/胚胎的质量提供了潜在的分子标记。在这篇综述中,我们概述了卵母细胞成熟、受精和早期胚胎发育过程中异常的遗传决定因素。目前,已有 16 个基因(PATL2、TUBB8、TRIP13、ZP1、ZP2、ZP3、PANX1、TLE6、WEE2、CDC20、BTG4、PADI6、NLRP2、NLRP5、KHDC3L 和 REC114)被报道为卵母细胞成熟阻滞、受精失败、胚胎阻滞和植入前胚胎致死的原因。这些异常主要具有孟德尔遗传模式,包括显性遗传和隐性遗传,尽管在某些情况下也出现了新生突变。在这篇综述中,我们将介绍每个基因在人类早期生殖特定过程中的作用,并总结这些基因中所有已知的变体及其相应的表型。一些基因的变体对人类早期生殖过程中的某些步骤有特定的影响,而其他变体则导致一系列表型。这些变体和遗传标记将为患者的个体化遗传咨询和潜在治疗奠定基础,并成为生殖医学中精准治疗的目标。