Kaleva Marko, Toppari Jorma
Department of Physiology, University of Turku, 20520 Turku, Finland.
Cell Tissue Res. 2005 Oct;322(1):167-72. doi: 10.1007/s00441-005-1143-3. Epub 2005 Nov 3.
Cryptorchidism is a common ailment of new-born boys, affecting 1-9% of full term boys at birth. Cryptorchidism has been associated with an increased risk of testicular cancer and reduced fertility. Aetiology of cryptorchidism remains obscure in most cases. Familial occurrence suggests a heritable susceptibility to cryptorchidism; however, seasonal variation in the incidence of cryptorchidism suggests that environmental factors also contribute. Testicular descent is characterised by androgen-dependent regression of cranial suspensory ligament and androgen + insulin-like hormone 3 (Ins l3)-dependent gubernacular outgrowth. Even though hormonal defects are rarely detected in patients, both hypo-and hypergonadotropic hormonal patterns have been associated with cryptorchidism. Moreover, cryptorchid boys have significantly reduced serum androgen bioactivity at 3 months of age when normal boys have a strong surge of reproductive hormones. Defects in Ins l3 action cause cryptorchidism in male mice, and over-expression in female mice causes ovarian descent. Defects in leucine-rich repeat-containing G-protein-coupled receptor 8/G-protein-coupled receptor affecting testis descent (LGR8/GREAT), the receptor for Ins l3, manifest the same phenotype as Ins l3 knockout mutants. Even though mutations found in Ins l3 and LGR8/GREAT genes are not a common cause of cryptorchidism in patients, it remains to be resolved whether low Ins l3 levels during development are associated with cryptorchidism. Cryptorchidism may reflect foetal testicular dysgenesis that may later manifest as subfertility or testicular cancer.
隐睾症是新生男婴的常见疾病,在足月出生的男婴中发病率为1% - 9%。隐睾症与睾丸癌风险增加和生育能力降低有关。在大多数情况下,隐睾症的病因仍不明确。家族性发病提示存在对隐睾症的遗传易感性;然而,隐睾症发病率的季节性变化表明环境因素也有影响。睾丸下降的特征是颅悬韧带的雄激素依赖性退化以及雄激素和胰岛素样激素3(Ins l3)依赖性的引带生长。尽管在患者中很少检测到激素缺陷,但低促性腺激素和高促性腺激素的激素模式都与隐睾症有关。此外,在正常男婴生殖激素大量激增的3个月时,隐睾男婴的血清雄激素生物活性显著降低。Ins l3作用缺陷会导致雄性小鼠患隐睾症,而在雌性小鼠中过表达则会导致卵巢下降。富含亮氨酸重复序列的G蛋白偶联受体8/影响睾丸下降的G蛋白偶联受体(LGR8/GREAT),即Ins l3的受体,其缺陷表现出与Ins l3基因敲除突变体相同的表型。尽管在Ins l3和LGR8/GREAT基因中发现的突变并非患者隐睾症的常见病因,但发育过程中Ins l3水平低是否与隐睾症有关仍有待解决。隐睾症可能反映了胎儿睾丸发育不全,这可能在以后表现为生育力低下或睾丸癌。