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雄激素不敏感综合征患者生殖器及耻骨上皮肤中继发性5α-还原酶缺乏的证据。

Evidence for secondary 5 alpha-reductase deficiency in genital and supra-pubic skin of subjects with androgen insensitivity syndrome.

作者信息

Wilson S C, Oakey R E, Scott J S

机构信息

Department of Chemical Pathology, University of Leeds, England.

出版信息

Acta Endocrinol (Copenh). 1988 Mar;117(3):353-60. doi: 10.1530/acta.0.1170353.

DOI:10.1530/acta.0.1170353
PMID:3164158
Abstract

The activity of 5 alpha-reductase in genital and supra-pubic skin (homogenate or fibroblasts) from subjects with complete or incomplete androgen insensitivity syndrome was low compared with mean activity in samples from normally differentiated male controls. Also, in two subjects with incomplete androgen insensitivity syndrome the ratio of the concentration of testosterone to that of 5 alpha-dihydrotestosterone in plasma was raised after hCG stimulation but normal under basal conditions. In three subjects with complete androgen insensitivity syndrome there was no evidence of raised ratios of testosterone to 5 alpha-dihydrotestosterone in plasma under basal or hCG-stimulated conditions. The activities of other steroid metabolizing enzymes, e.g. 17 beta-hydroxysteroid dehydrogenase, 3 alpha/beta-hydroxysteroid dehydrogenase, were not decreased. The low 5 alpha-reductase activity of androgen insensitive subjects reported here, and by others, may imply that this enzyme in genital skin is in some way androgen dependent, or responsive to other factors associated with androgen insensitivity syndrome.

摘要

与正常分化男性对照组样本的平均活性相比,完全或不完全雄激素不敏感综合征患者生殖器及耻骨上皮肤(匀浆或成纤维细胞)中5α-还原酶的活性较低。此外,两名不完全雄激素不敏感综合征患者在人绒毛膜促性腺激素(hCG)刺激后,血浆中睾酮浓度与5α-双氢睾酮浓度的比值升高,但在基础条件下正常。在三名完全雄激素不敏感综合征患者中,无论是基础条件还是hCG刺激条件下,均未发现血浆中睾酮与5α-双氢睾酮的比值升高。其他类固醇代谢酶的活性,如17β-羟类固醇脱氢酶、3α/β-羟类固醇脱氢酶,并未降低。此处及其他研究报道的雄激素不敏感患者5α-还原酶活性较低,可能意味着生殖器皮肤中的这种酶在某种程度上依赖雄激素,或对与雄激素不敏感综合征相关的其他因素有反应。

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