The effect of 5α-reductase-2 deficiency on human fertility.
The effect of 5α-reductase-2 deficiency on human fertility.
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5α-还原酶-2缺乏对人类生育的影响。
DOI：
10.1016/j.fertnstert.2013.11.128
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发表时间：
2014-02
影响因子：
6.7
通讯作者：
Rosenwaks, Zev
中科院分区：
文献类型：
--
作者：
Kang, Hey-Joo;Imperato-McGinley, Julianne;Zhu, Yuan-Shan;Rosenwaks, Zev
最有趣和耐人寻味的男性性别分化障碍是由于5-α-还原酶-2同工酶缺乏。这些男性由于缺乏催化睾酮转化为双氢睾酮(DHT)的能力而出生时外生殖器不明确。DHT是一种有效的雄激素，负责将泌尿生殖窦和生殖器结节分化为外生殖器、尿路和前列腺。受影响的男性出生时有阴蒂样、阴囊二裂、尿道下裂、因不完全闭合泌尿生殖窦而导致的盲性浅阴道袋和发育不全的前列腺。在青春期，主要是睾丸素的产生激增促进了男性化，导致大多数人选择将性别重新分配给男性。对于受影响的男性来说，生育能力是一个挑战，原因有几个。未矫正的隐睾症与低精子产量有关，而且有证据表明精原细胞向精母细胞的转化存在缺陷。不发达的前列腺和随之而来的低精液量会影响精子的运输。此外，由于缺乏前列腺特异性抗原，精液可能不会液化。在这篇综述中，我们讨论了5-α-还原酶-2缺乏综合征及其对人类生育的影响。
A most interesting and intriguing male disorder of sexual differentiation is due to 5α-reductase-2 isoenzyme deficiency. These males are born with ambiguous external genitalia due to a deficiency in their ability to catalyze the conversion of testosterone to dihydrotestosterone (DHT). DHT is a potent androgen responsible for differentiation of the urogenital sinus and genital tubercle into the external genitalia, urethra and prostate. Affected males are born with a clitoral-like phallus, bifid scrotum, hypospadias, blind shallow vaginal pouch from incomplete closure of the urogenital sinus and a rudimentary prostate. At puberty, the surge in mainly testosterone production prompts virilization, causing most to choose gender reassignment to male. Fertility is a challenge for affected men for several reasons. Uncorrected cryptorchidism is associated with low sperm production, and there is evidence of defective transformation of spermatogonia into spermatocytes. The underdeveloped prostate and consequent low semen volumes affect sperm transport. Additionally, semen may not liquefy due to a lack of prostate-specific antigen. In this review, we discuss the 5α-reductase-2 deficiency syndrome and its impact on human fertility.
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影响因子：
158.5
作者：
IMPERATOMCGINLEY, J;PETERSON, RE;STURLA, E
通讯作者：
STURLA, E
影响因子：
5.8
作者：
IMPERATOMCGINLEY, J;GAUTIER, T;KAZAM, E
通讯作者：
KAZAM, E
DOI：
10.1196/annals.1336.023
发表时间：
2005-01-01
期刊：
TESTICULAR CELL DYNAMICS AND ENDOCRINE SIGNALING
影响因子：
--
作者：
Jarow, JP;Zirkin, BR
通讯作者：
Zirkin, BR
影响因子：
--
作者：
FRATIANNI, CM;IMPERATOMCGINLEY, J
通讯作者：
IMPERATOMCGINLEY, J
影响因子：
6.6
作者：
Hadziselimovic, F.;Dessouky, N.
通讯作者：
Dessouky, N.