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三名婴儿睾丸的体外类固醇生成研究,其中两名外生殖器模糊,一名真性性早熟:未成熟人类睾丸中存在活性17β-羟类固醇氧化还原酶的证据。

In vitro steroidogenesis in testes of three infants, two with ambiguous external genitalia and one with true precocious puberty: Evidence for the presence of active 17 beta-hydroxysteroid oxidoreductase in immature human testes.

作者信息

Oshima H, Ishiwata D, Hatakeyama S, Yokokawa M

出版信息

Endocrinol Jpn. 1980 Feb;27(1):59-64. doi: 10.1507/endocrj1954.27.59.

DOI:10.1507/endocrj1954.27.59
PMID:6248335
Abstract

The present report investigated steroidogenesis in vitro in testis tissues obtained from two boys aged 8 months and 4 years with ambiguous external genitalia and male vagina, and a 4-year-old body with true precocious puberty. Histologically, testes of the former two boys are still immature and the testis of the last one contains differentiated Sertoli cells and primary spermatocytes, but no mature Leydig cells are recognized in any of them. In each testis, 17 beta-hydroxysteroid oxidoreductase is active for androstenedione in the presence of an excess amount of NADPH, while delta 5-3 beta-hydroxysteroid dehydrogenase and delta 4-steroid 5 alpha-reductase activities are limited. 17 alpha-Hydroxylase and C17--20 lyase are significantly active in each testis and are enhanced in the testis of the boy with precocious puberty. Although the testis tissue used in the present study may not be biologically normal and the number of cases investigated is still limited, the above results indicate that active 17 beta-hydroxysteroid oxidoreductase is present in immature human testes and that delta 5-3 beta-hydroxysteroid dehydrogenase may become active in the human testis at the advanced stage of the development of testicular function during the puberty.

摘要

本报告研究了从两名患有外生殖器模糊和男性阴道的8个月大及4岁男孩以及一名患有真性性早熟的4岁男孩身上获取的睾丸组织的体外类固醇生成。组织学上,前两名男孩的睾丸仍未成熟,最后一名男孩的睾丸含有分化的支持细胞和初级精母细胞,但在任何一个睾丸中均未识别出成熟的睾丸间质细胞。在每个睾丸中,在过量的NADPH存在下,17β-羟基类固醇氧化还原酶对雄烯二酮具有活性,而δ5-3β-羟基类固醇脱氢酶和δ4-类固醇5α-还原酶的活性有限。17α-羟化酶和C17-20裂解酶在每个睾丸中均具有显著活性,并且在性早熟男孩的睾丸中活性增强。尽管本研究中使用的睾丸组织可能并非生物学上正常的,且所研究的病例数量仍然有限,但上述结果表明,活性17β-羟基类固醇氧化还原酶存在于未成熟的人类睾丸中,并且δ5-3β-羟基类固醇脱氢酶可能在青春期睾丸功能发育的晚期在人类睾丸中变得活跃。

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In vitro steroidogenesis in testes of three infants, two with ambiguous external genitalia and one with true precocious puberty: Evidence for the presence of active 17 beta-hydroxysteroid oxidoreductase in immature human testes.三名婴儿睾丸的体外类固醇生成研究,其中两名外生殖器模糊,一名真性性早熟:未成熟人类睾丸中存在活性17β-羟类固醇氧化还原酶的证据。
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