New Studies of the llβ-Hydroxylase and 18-Hydroxylase Enzymes in the Hypertensive Form of Congenital Adrenal Hyperplasia*
New Studies of the llβ-Hydroxylase and 18-Hydroxylase Enzymes in the Hypertensive Form of Congenital Adrenal Hyperplasia*
复制标题
先天性肾上腺增生高血压中 11β-羟化酶和 18-羟化酶的新研究*
DOI：
--
复制
发表时间：
1980
期刊：
影响因子：
--
通讯作者：
M. New
中科院分区：
文献类型：
--
作者：
L. Levine;W. Rauh;K. Gottesdiener;D. Chow;P. Gunczler,;R. Rapaport;S. Pang;B. Schneider;M. New
对4例11β-羟化酶缺乏所致先天性肾上腺增生儿童的研究提供了证据，证明束状肾上腺和球状肾上腺中存在两个独立的11β-羟化系统。此外，这些研究支持了肾上腺11β-和18-羟化活性相关并且可能涉及相同的酶蛋白和催化位点的提议。在未治疗或治疗不良的状态下，尽管脱氧皮质酮（DOC）和四氢脱氧皮质酮升高，但尿游离18-羟基-DOC处于正常范围内，并且不随ACTH正常增加。PRA和尿游离18-羟皮质酮（18-OHB），四氢醛固酮（TH Aldo），和pH 1醛固酮在未经处理和ACTH期间被抑制。糖皮质激素的管理抑制血浆ACTH，尿四氢DOC和游离DOC排泄减少到正常范围。结论：尿中18-OHB、TH、Aldo、pH 1ald水平与PRA呈负相关，尿中PRA水平与尿中18-OHB、TH、Aldo、pH 1ald水平呈负相关。
Studies in four children with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency provide evidence for two separate 11β-hydroxylating systems in the adrenal zona fasciculata and zona glomerulosa. In addition, these studies support the proposal that the adrenal 11β- and 18-hydroxylating activities are related and may involve the same enzyme protein and catalytic site. In the untreated or poorly treated state, despite elevation of deoxycorticosterone (DOC) and tetrahydrodeoxycorticosterone, urinary free 18-hydroxy-DOC was in the low normal range and did not increase normally with ACTH. PRA and urinary free 18-hydroxycorticosterone (18-OHB), tetrahydroaldosterone (TH Aldo), and pH 1 aldosterone were suppressed in the untreated and ACTH periods. Glucocorticoid administration suppressed plasma ACTH, and urinary tetrahydro-DOC and free DOC excretion decreased to the normal range. Concomitantly, there was a rise in PRA accompanied by parallel increase in urinary 18-OHB, urinary TH Aldo, and pH 1 ald...