van der Ven K, Messer L, van der Ven H, Jeyendran R S, Ober C
Department of Obstetrics and Gynecology, University of Bonn, Germany.
Hum Reprod. 1996 Mar;11(3):513-7. doi: 10.1093/humrep/11.3.513.
The majority of men with cystic fibrosis (CF) are infertile due to a bilateral congenital absence of the vas deferens (CBAVD). However, clinically affected CF patients present a spectrum of genital phenotypes ranging from normal fertility to severely impaired spermatogenesis and CBAVD. Recently, it has become apparent that CF can manifest itself as isolated CBAVD in the absence of other clinical symptoms. The present study was undertaken to test the possible involvement of the CF gene in the aetiology of male infertility other than CBAVD. Semen specimens from 127 unrelated healthy males with various diagnoses of reduced sperm quality were screened for a panel of 13 mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Fourteen of 80 (17.5%) healthy men with infertility due to reduced sperm quality and 3 of 21 (14.3%) men with azoospermia had at least one CF mutation (one azoospermic male was a compound heterozygote). The frequency of mutations in our sample of infertile males was significantly higher than the expected CF carrier frequency in the local population (P = 0.00139). No mutations were found in a control group of 26 individuals with normal semen parameters. This increased frequency of CF mutations in healthy men with reduced sperm quality and in men with azoospermia without CBAVD suggests that the CFTR protein may be involved in the process of spermatogenesis or sperm maturation apart from playing a critical role in the development of the epididymal glands and the vas deferens.
大多数患有囊性纤维化(CF)的男性因双侧先天性输精管缺如(CBAVD)而不育。然而,临床上受影响的CF患者表现出一系列生殖表型,从正常生育能力到严重受损的精子发生和CBAVD。最近,很明显CF可在无其他临床症状的情况下表现为孤立的CBAVD。本研究旨在测试CF基因在除CBAVD之外的男性不育病因中可能的作用。对127名诊断为精子质量下降的无关健康男性的精液标本进行筛查,检测囊性纤维化跨膜传导调节因子(CFTR)基因中的一组13个突变。80名因精子质量下降导致不育的健康男性中有14名(17.5%),21名无精子症男性中有3名(14.3%)至少有一个CF突变(一名无精子症男性为复合杂合子)。我们不育男性样本中的突变频率显著高于当地人群中预期的CF携带者频率(P = 0.00139)。在26名精液参数正常的个体组成的对照组中未发现突变。精子质量下降的健康男性和无CBAVD的无精子症男性中CF突变频率的增加表明,CFTR蛋白除了在附睾和输精管的发育中起关键作用外,可能还参与精子发生或精子成熟过程。