Darko Michelle E, Kappy Michelle, Rabizadeh Daniel, Jalas Chaim, Forman Eric J, Brady Paula, Williams Zev
Department of Obstetrics and Gynecology, Columbia University Irving Medical Center, New York, NY, USA.
Columbia University Fertility Center, Department of Obstetrics and Gynecology, Columbia University Irving Medical Center, New York, NY, USA.
HGG Adv. 2025 Apr 21;6(3):100443. doi: 10.1016/j.xhgg.2025.100443.
Women with genetic causes of infertility are more likely to experience recurrent pregnancy loss (RPL). Advances in whole-genome sequencing (WGS) have allowed for the improved detection of such genes. One reproductively young patient with a history of RPL underwent 5 in vitro fertilization cycles with nearly complete arrest of blastocyst development and ubiquitous aneuploidy of maternal origin in arrested embryos. Here, we present the discovery of a gene variant, RNF212B, as a potential genetic cause of female infertility and RPL. DNA was extracted and submitted for WGS. After filtering out variants with Genome Aggregation Database allele frequencies exceeding 0.25%, we identified 87 unique variants and conducted a literature search to identify potential associations with infertility. PGT-A analysis of arrested embryos revealed extensive aneuploidies affecting many chromosomes in all embryos. Maternal WGS revealed a homozygous stop-gain mutation in the RNF212B gene. RNF212 has been shown to interact with proteins involved in meiotic recombination, including DMC1 and DNA repair protein RAD51. This homozygous nonsense mutation in the RNF212B gene may be responsible for the presence of aberrant oogonium and for disrupting the meiotic recombination process, thereby contributing to female infertility and RPL.
因遗传因素导致不孕的女性更易经历复发性流产(RPL)。全基因组测序(WGS)技术的进步使得此类基因的检测得以改进。一位有复发性流产病史的年轻育龄患者接受了5次体外受精周期,囊胚发育几乎完全停滞,且停滞胚胎中普遍存在母源性非整倍体。在此,我们报告发现一种基因变异体RNF212B,它可能是女性不孕和复发性流产的潜在遗传原因。提取DNA并进行全基因组测序。在滤除基因组聚合数据库等位基因频率超过0.25%的变异体后,我们鉴定出87个独特变异体,并进行文献检索以确定与不孕的潜在关联。对停滞胚胎的植入前遗传学检测分析显示,所有胚胎中许多染色体都存在广泛的非整倍体。母亲的全基因组测序显示RNF212B基因存在纯合性的终止密码子获得性突变。RNF212已被证明与参与减数分裂重组的蛋白质相互作用,包括DMC1和DNA修复蛋白RAD51。RNF212B基因的这种纯合无义突变可能是异常卵原细胞存在的原因,并导致减数分裂重组过程中断,从而导致女性不孕和复发性流产。