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FANCM 基因突变致男性单纯型支持细胞综合征伴弥漫性星形细胞瘤 1 例

FANCM Gene Variants in a Male Diagnosed with Sertoli Cell-Only Syndrome and Diffuse Astrocytoma.

机构信息

Genom Ltd., Ilica 190, 10000 Zagreb, Croatia.

Department of Medical Biology, School of Medicine, University of Zagreb, Šalata 3, 10000 Zagreb, Croatia.

出版信息

Genes (Basel). 2024 May 28;15(6):707. doi: 10.3390/genes15060707.

DOI:10.3390/genes15060707
PMID:38927643
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11202954/
Abstract

Azoospermia is a form of male infertility characterized by a complete lack of spermatozoa in the ejaculate. Sertoli cell-only syndrome (SCOS) is the most severe form of azoospermia, where no germ cells are found in the tubules. Recently, FANCM gene variants were reported as novel genetic causes of spermatogenic failure. At the same time, FANCM variants are known to be associated with cancer predisposition. We performed whole-exome sequencing on a male patient diagnosed with SCOS and a healthy father. Two compound heterozygous missense mutations in the FANCM gene were found in the patient, both being inherited from his parents. After the infertility assessment, the patient was diagnosed with diffuse astrocytoma. Immunohistochemical analyses in the testicular and tumor tissues of the patient and adequate controls showed, for the first time, not only the existence of a cytoplasmic and not nuclear pattern of FANCM in astrocytoma but also in non-mitotic neurons. In the testicular tissue of the SCOS patient, cytoplasmic anti-FANCM staining intensity appeared lower than in the control. Our case report raises a novel possibility that the infertile carriers of FANCM gene missense variants could also be prone to cancer development.

摘要

无精子症是一种男性不育症,其特征是精液中完全缺乏精子。唯支持细胞综合征(SCOS)是无精子症最严重的形式,在小管中未发现生殖细胞。最近,FANCM 基因突变被报道为精子发生失败的新的遗传原因。同时,已知 FANCM 变体与癌症易感性有关。我们对一名被诊断为 SCOS 的男性患者和一名健康父亲进行了全外显子组测序。在患者中发现了 FANCM 基因中的两个复合杂合错义突变,均遗传自他的父母。在进行不育评估后,该患者被诊断为弥漫性星形细胞瘤。患者和适当对照的睾丸和肿瘤组织的免疫组织化学分析首次表明,FANCM 不仅存在于星形细胞瘤中细胞质而不是核中,而且存在于非有丝分裂神经元中。在 SCOS 患者的睾丸组织中,细胞质抗 FANCM 染色强度似乎低于对照。我们的病例报告提出了一种新的可能性,即 FANCM 基因突变的不育携带者也可能容易发生癌症发展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/6e2c19a4849f/genes-15-00707-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/4c53cf17662e/genes-15-00707-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/3407068c6809/genes-15-00707-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/6e2c19a4849f/genes-15-00707-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/4c53cf17662e/genes-15-00707-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/3407068c6809/genes-15-00707-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/11e7/11202954/6e2c19a4849f/genes-15-00707-g003.jpg

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