Uddin Islam, Zafar Iqra, Xu Caoling, Li Wenqing, Khan Muhammad Imran, Wu Limin, Bao Jianqiang
Center for Reproduction and Genetics, Division of Life Sciences and Medicine, The First Affiliated Hospital of USTC, University of Science and Technology of China, Hefei, Anhui, 230001, China.
Center for Advanced Interdisciplinary Science and Biomedicine of IHM, Biomedical Sciences and Health Laboratory of Anhui Province, Hefei National Laboratory for Physical Sciences at Microscale, University of Science and Technology of China (USTC), Hefei, Anhui, China.
Mol Genet Genomics. 2024 Dec 3;299(1):111. doi: 10.1007/s00438-024-02205-7.
Azoospermia, a severe form of male infertility characterized by the complete absence of sperm in the ejaculate, affects about 1% of the male population, with most cases attributed to nonobstructive azoospermia (NOA) caused by gametogenic failure. NOA has various genetic origins, including chromosomal abnormalities, Y chromosome microdeletions, and monogenic mutations. Although whole-exome sequencing (WES) has identified over thirty candidate genes associated with NOA, the genetic causes of most cases have yet to be elucidated. In our study, we selected seven consanguineous families diagnosed with azoospermia from a total of 21 male infertile families recruited from the rural area of Pakistan. Blood samples were collected from both patients and fertile controls for DNA extraction, followed by WES to identify potential causative recessive monogenic variants linked to male infertility. We successfully identified five deleterious variants among five of the seven families, including three missense biallelic substitutions in WWC2, RPL10L, and SOHLH1, a hemizygous deletion in ESX1, and a homozygous deletion in TXNDC2, which have potentially pathogenic relevance to the azoospermia of human male infertility. These novel findings enhance our understanding of the molecular mechanisms underlying the complex etiology of azoospermia, offering valuable insights for genetic counseling and diagnostics and paving the way for future therapeutic approaches.
无精子症是男性不育的一种严重形式,其特征是射精中完全没有精子,影响约1%的男性人口,大多数病例归因于由生精功能衰竭引起的非梗阻性无精子症(NOA)。NOA有多种遗传起源,包括染色体异常、Y染色体微缺失和单基因变异。尽管全外显子组测序(WES)已经鉴定出三十多个与NOA相关的候选基因,但大多数病例的遗传原因仍有待阐明。在我们的研究中,我们从巴基斯坦农村地区招募的21个男性不育家庭中选择了7个被诊断为无精子症的近亲家庭。从患者和可育对照中采集血液样本进行DNA提取,然后进行WES以鉴定与男性不育相关的潜在致病隐性单基因变异。我们在7个家庭中的5个家庭中成功鉴定出5个有害变异,包括WWC2、RPL10L和SOHLH1中的3个错义双等位基因替代、ESX1中的一个半合子缺失以及TXNDC2中的一个纯合子缺失,这些变异与人类男性不育的无精子症具有潜在的致病相关性。这些新发现增强了我们对无精子症复杂病因背后分子机制的理解,为遗传咨询和诊断提供了有价值的见解,并为未来的治疗方法铺平了道路。