Li Lin, Sha Yan-Wei, Su Zhi-Ying, Mei Li-Bin, Ji Zhi-Yong, Zhang Qing, Lin Shao-Bin, Wang Xu, Qiu Ping-Ping, Li Ping, Yin Chenghong
Central Laboratory, Beijing Obstetrics and Gynecology Hospital, Capital Medical University, Chaoyang, Beijing 100026, China.
Department of Reproductive Medicine, Xiamen Maternity and Child Care Hospital, Xiamen, Fujian 361005, China.
Gene. 2018 Jan 10;639:106-110. doi: 10.1016/j.gene.2017.10.014. Epub 2017 Oct 7.
Severe oligozoospermia (SO) is a common disease resulting in male infertility; however, its pathophysiology remains unclear. Here, we report two brothers with SO. Whole-exome sequencing (WES) identified a hemizygous variant in HAUS7 (c.G386T:p.G129V), an X-linked gene. HAUS7 has been reported to play a role in the meiotic maturation and chromosome alignment of germ cells. The two patients inherited this variant from their mother, and this variant was considered to be a highly pathogenic mutation by in silico analysis. Moreover, in vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI) was carried out in both the proband's wife and the brother's wife, but they failed to become pregnant after the embryo transfers. Therefore, this novel mutation in HAUS7 gene may be associated with severe oligozoospermia.
严重少精子症(SO)是导致男性不育的常见疾病;然而,其病理生理学仍不清楚。在此,我们报告了两名患有严重少精子症的兄弟。全外显子组测序(WES)在一个X连锁基因HAUS7中鉴定出一个半合子变异(c.G386T:p.G129V)。据报道,HAUS7在生殖细胞的减数分裂成熟和染色体排列中起作用。这两名患者从他们的母亲那里遗传了这个变异,并且通过计算机分析,这个变异被认为是一个高致病性突变。此外,先证者的妻子和其兄弟的妻子都接受了体外受精(IVF)/卵胞浆内单精子注射(ICSI),但在胚胎移植后均未怀孕。因此,HAUS7基因中的这种新突变可能与严重少精子症有关。