Mason J I, Hemsell P G
Endocrinology. 1982 Jul;111(1):208-13. doi: 10.1210/endo-111-1-208.
The metabolism of cholesterol sulfate (5-cholesten-3 beta-ol sodium sulfate) and cholesterol to pregnenolone sulfate and pregnenolone, respectively, in human fetal adrenal mitochondria has been investigated. Cholesterol sulfate (50 microM) was converted efficiently to pregnenolone sulfate (5-pregnen-3 beta-ol-20-one sodium sulfate; 1.6 nmol min-2 mg-1 protein). Pregnenolone sulfate biosynthesis from endogenous mitochondrial precursors could not be detected. The rate of pregnenolone biosynthesis in the presence of cholesterol (50 microM) was 0.2 nmol min-1 mg-1 protein. In contrast, mitochondria obtained from the adrenal tissue of a boy and two adult men did not produce detectable amounts of pregnenolone sulfate after the addition of cholesterol sulfate (50 microM). Pregnenolone was produced efficiently by adrenal mitochondria from endogenous mitochondrial precursors and was not stimulated by the addition of cholesterol (100 microM). The results suggest that human fetal adrenal mitochondria contain a specific cholesterol sulfate desmolase and that cholesterol sulfate may be the predominant sterol substrate for the large amounts of steroid sulfates produced by this tissue. Whereas the metabolism of cholesterol to form pregnenolone was increased by the addition of 1 mM calcium ions, the metabolism of cholesterol sulfate to produce pregnenolone sulfate was not stimulated by calcium ions. This latter finding may indicate that the metabolism of cholesterol sulfate in the human fetal adrenal gland is controlled separately from the metabolism of cholesterol.
已对人胎儿肾上腺线粒体中硫酸胆固醇（5-胆甾烯-3β-醇硫酸钠）和胆固醇分别代谢生成硫酸孕烯醇酮和孕烯醇酮的过程进行了研究。硫酸胆固醇（50微摩尔）能有效地转化为硫酸孕烯醇酮（5-孕烯-3β-醇-20-酮硫酸钠；1.6纳摩尔·分钟⁻¹·毫克⁻¹蛋白质）。未检测到由内源性线粒体前体合成硫酸孕烯醇酮的情况。在存在胆固醇（50微摩尔）的情况下，孕烯醇酮的合成速率为0.2纳摩尔·分钟⁻¹·毫克⁻¹蛋白质。相比之下，在加入硫酸胆固醇（50微摩尔）后，从一名男孩和两名成年男性的肾上腺组织获得的线粒体未产生可检测量的硫酸孕烯醇酮。肾上腺线粒体能有效地由内源性线粒体前体产生孕烯醇酮，且添加胆固醇（100微摩尔）不会对其产生刺激作用。结果表明，人胎儿肾上腺线粒体含有一种特定的硫酸胆固醇碳链裂解酶，且硫酸胆固醇可能是该组织产生大量甾体硫酸盐的主要甾醇底物。虽然加入1毫摩尔钙离子会增加胆固醇代谢生成孕烯醇酮的量，但钙离子不会刺激硫酸胆固醇代谢生成硫酸孕烯醇酮。后一发现可能表明，人胎儿肾上腺中硫酸胆固醇的代谢与胆固醇的代谢是分开控制的。
