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全外显子组测序在来自巴基斯坦近亲家庭的无精子症患者中鉴定出罕见的隐性变异。

Whole-exome sequencing identifies rare recessive variants in azoospermia patients from consanguineous Pakistani families.

作者信息

Uddin Islam, Zafar Iqra, Xu Caoling, Li Wenqing, Khan Muhammad Imran, Wu Limin, Bao Jianqiang

机构信息

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.

出版信息

Mol Genet Genomics. 2024 Dec 3;299(1):111. doi: 10.1007/s00438-024-02205-7.

DOI:10.1007/s00438-024-02205-7
PMID:39625557
Abstract

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.

摘要

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。

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本文引用的文献

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Andrology. 2022 Nov;10(8):1605-1624. doi: 10.1111/andr.13269. Epub 2022 Sep 7.
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巴基斯坦血缘婚姻及其与妇女生殖健康和生育行为的关系:1990-2018 年人口与健康调查的二次数据分析。
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AlphaFold Protein Structure Database: massively expanding the structural coverage of protein-sequence space with high-accuracy models.AlphaFold 蛋白质结构数据库:用高精度模型极大地扩展蛋白质序列空间的结构覆盖范围。
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The mutation c.346-1G > A in SOHLH1 impairs sperm production in the homozygous but not in the heterozygous condition.SOHLH1 基因中的 c.346-1G > A 突变纯合时会导致精子生成受损,但杂合时不会。
Hum Mol Genet. 2022 Mar 31;31(7):1013-1021. doi: 10.1093/hmg/ddab242.
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Highly accurate protein structure prediction with AlphaFold.利用 AlphaFold 进行高精度蛋白质结构预测。
Nature. 2021 Aug;596(7873):583-589. doi: 10.1038/s41586-021-03819-2. Epub 2021 Jul 15.
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TXNDC2 joint molecular marker is associated with testis pathology and is an accurate predictor of sperm retrieval.TXNDC2 联合分子标志物与睾丸病变相关,是精子获取的准确预测指标。
Sci Rep. 2021 Jun 22;11(1):13064. doi: 10.1038/s41598-021-92603-3.
8
Association of ESX1 gene variants with non-obstructive azoospermia in Chinese males.ESX1 基因突变与中国男性非梗阻性无精子症的相关性研究。
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The Hippo pathway component Wwc2 is a key regulator of embryonic development and angiogenesis in mice.Hippo 通路组件 Wwc2 是小鼠胚胎发育和血管生成的关键调节因子。
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