Trezise A E, Linder C C, Grieger D, Thompson E W, Meunier H, Griswold M D, Buchwald M
Department of Genetics, Hospital for Sick Children, Toronto, Ontario, Canada.
Nat Genet. 1993 Feb;3(2):157-64. doi: 10.1038/ng0293-157.
Severely reduced fertility is a common finding in cystic fibrosis (CF). We used in situ hybridization to examine the cell-specific expression of CFTR in the reproductive organs of rodents. In males CFTR mRNA is found in the round spermatids (spermatogenic stages V-X) and in the principal cells that line the initial segment of the epididymis. In both the testis and the epididymis, CFTR expression is developmentally regulated suggesting that the defect in the genital tract of male CF patients is of developmental origin. CFTR expression in the luminal and glandular epithelium of the uterus is regulated during the oestrous cycle and is maximal at pro-oestrus. Our results provide a biological rationale for the reduced fertility of CF patients, and suggest a possible cause for the comparatively poorer prognosis for women with CF.
严重生育力下降是囊性纤维化（CF）的常见表现。我们采用原位杂交技术检测CFTR在啮齿动物生殖器官中的细胞特异性表达。在雄性动物中，CFTR mRNA存在于圆形精子细胞（生精阶段V - X）以及附睾起始段内衬的主细胞中。在睾丸和附睾中，CFTR表达受发育调控，这表明男性CF患者生殖道的缺陷源于发育过程。子宫腔上皮和腺上皮中的CFTR表达在发情周期中受到调控，在发情前期达到最大值。我们的研究结果为CF患者生育力下降提供了生物学依据，并提示了CF女性患者预后相对较差的可能原因。
