Liu Lingyun, He Yan, Guo Kaimin, Zhou Linying, Li Xian, Tseng Michael, Cai Lu, Lan Zi-Jian, Zhou Junmei, Wang Hongliang, Lei Zhenmin
Department of Andrology, The First Hospital of Jilin University, Changchun, China.
Department of Obstetrics, Gynecology and Women's Health, University of Louisville School of Medicine, Louisville, Kentucky.
Am J Pathol. 2017 Nov;187(11):2508-2519. doi: 10.1016/j.ajpath.2017.07.016. Epub 2017 Aug 18.
Gametogenetin binding protein 2 (GGNBP2) is an evolutionarily conserved zinc finger protein. Although Ggnbp2-null embryos in the B6 background died because of a defective placenta, 6.8% of Ggnbp2-null mice in the B6/129 mixed background were viable and continued to adulthood. Adult Ggnbp2-null males were sterile, with smaller testes and an azoospermic phenotype, whereas mutant females were fertile. Histopathological analysis of 2-month-old Ggnbp2-null testes revealed absence of mature spermatozoa in the seminiferous tubules and epididymides and reduction of the number of spermatids. Ultrastructural analysis indicated dramatic morphological defects of developing spermatids in the Ggnbp2-null testes, including irregularly shaped acrosomes, acrosome detachment, cytoplasmic remnant, ectopic manchette, and ill-formed head shape in both elongating and elongated spermatids. However, the numbers of spermatogonia, spermatocytes, Leydig cells, and Sertoli cells in Ggnbp2-null testes did not significantly differ from the wild-type siblings. Gonadotropins, testosterone, and the blood-testis barrier were essentially unaffected. Western blot analyses showed increases in α-E-catenin, β-catenin, and N-cadherin, decreases in E-cadherin, afadin, and nectin-3, and no changes in vinculin, nectin-2, focal adhesion kinase, and integrin-β1 protein levels in Ggnbp2-null testes compared to wild-type siblings. Together, this study demonstrates that GGNBP2 is critically required for maintenance of the adhesion integrity of the adlumenal germ epithelium and is indispensable for normal spermatid transformation into mature spermatozoa in mice.
配子生成素结合蛋白2(GGNBP2)是一种进化保守的锌指蛋白。尽管B6背景下的Ggnbp2基因敲除胚胎因胎盘缺陷而死亡,但在B6/129混合背景下,6.8%的Ggnbp2基因敲除小鼠存活至成年。成年Ggnbp2基因敲除雄性小鼠不育,睾丸较小且表现为无精子症,而突变雌性小鼠可育。对2月龄Ggnbp2基因敲除小鼠睾丸的组织病理学分析显示,生精小管和附睾中没有成熟精子,精子细胞数量减少。超微结构分析表明,Ggnbp2基因敲除小鼠睾丸中发育中的精子细胞存在明显的形态缺陷,包括顶体形状不规则、顶体脱离、细胞质残余、异位袖套以及伸长和已伸长精子细胞头部形状异常。然而,Ggnbp2基因敲除小鼠睾丸中的精原细胞、精母细胞、睾丸间质细胞和支持细胞数量与野生型同胞相比无显著差异。促性腺激素、睾酮和血睾屏障基本未受影响。蛋白质免疫印迹分析显示,与野生型同胞相比,Ggnbp2基因敲除小鼠睾丸中α-E-连环蛋白、β-连环蛋白和N-钙黏蛋白增加,E-钙黏蛋白、afadin和nectin-3减少,而纽蛋白、nectin-2、粘着斑激酶和整合素-β1蛋白水平无变化。总之,本研究表明,GGNBP2对于维持管腔上皮生殖细胞的黏附完整性至关重要,并且对于小鼠精子细胞正常转化为成熟精子不可或缺。