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Defects of steroidogenesis.

机译:类固醇生成的缺陷。

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摘要

In the biosynthesis of steroid hormones the neutral lipid cholesterol, a normal constituent of lipid bilayers is transformed via a series of hydroxylation, oxidation, and reduction steps into a vast array of biologically active compounds: mineralocorticoids, glucocorticoids, and sex hormones. Glucocorticoids regulate many aspects of metabolism and immune function, whereas mineralocorticoids help maintain blood volume and control renal excretion of electrolytes. Sex hormones are essential for sex differentiation in male and support reproduction. They include androgens, estrogens, and progestins. A block in the pathway of steroid biosynthesis leads to the lack of hormones downstream and accumulation of the upstream compounds that can activate other members of the steroid receptor family. This review deals with the clinical consequences of these blocks.
机译:在类固醇激素的生物合成中,中性脂质胆固醇(脂质双层的正常成分)通过一系列羟基化、氧化和还原步骤转化为大量生物活性化合物:盐皮质激素、糖皮质激素和性激素。糖皮质激素调节新陈代谢和免疫功能的许多方面,而盐皮质激素有助于维持血容量和控制肾脏排泄电解质。性激素对于男性的性别分化和支持生殖至关重要。它们包括雄激素、雌激素和孕激素。类固醇生物合成途径中的阻断导致下游激素的缺乏和上游化合物的积累,这些化合物可以激活类固醇受体家族的其他成员。本综述涉及这些阻滞的临床后果。

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