Illingworth D R, Corbin D K, Kemp E D, Keenan E J
Proc Natl Acad Sci U S A. 1982 Nov;79(21):6685-9. doi: 10.1073/pnas.79.21.6685.
Progesterone synthesis by the human corpus luteum requires a source of cholesterol, which can be derived from both local synthesis and uptake of low density lipoproteins (LDL). When the corpus luteum is maintained in organ culture, progesterone synthesis is primarily dependent on LDL and the rate of progesterone production during growth in a LDL-free media is suboptimal. An in vivo situation analogous to that of corpus luteum grown in LDL-depleted media exists naturally in patients with abetalipoproteinemia. To determine whether a complete deficiency of plasma LDL affects serum concentrations of progesterone (particularly during the luteal phase) or those of other hormones, we have measured the serum concentrations of luteinizing hormone, follicle-stimulating hormone, prolactin, estradiol, estrone, and progesterone during the menstrual cycle in a patient with phenotypic abetalipoproteinemia (on the basis of homozygous hypobetalipoproteinemia). Our results show a normal cyclical pattern with midcycle increases in the concentrations of luteinizing and follicle-stimulating hormones, prolactin, and estrogens but a distinctly subnormal increase in the luteal phase concentrations of progesterone. These results suggest that, in patients with phenotypic abetalipoproteinemia, the absence of LDL leads to an impairment in the maximal rates of production of progesterone by the corpus luteum.
人黄体合成孕酮需要胆固醇来源，胆固醇可来自局部合成和低密度脂蛋白（LDL）的摄取。当黄体维持在器官培养中时，孕酮合成主要依赖于LDL，并且在无LDL培养基中生长期间孕酮的产生速率不理想。无β脂蛋白血症患者自然存在一种类似于在LDL缺乏培养基中生长的黄体的体内情况。为了确定血浆LDL的完全缺乏是否会影响孕酮（特别是在黄体期）或其他激素的血清浓度，我们测量了一名表型无β脂蛋白血症患者（基于纯合子低β脂蛋白血症）月经周期中黄体生成素、卵泡刺激素、催乳素、雌二醇、雌酮和孕酮的血清浓度。我们的结果显示出正常的周期性模式，黄体生成素、卵泡刺激素、催乳素和雌激素的浓度在月经周期中期增加，但孕酮在黄体期的浓度明显低于正常增加。这些结果表明，在表型无β脂蛋白血症患者中，LDL的缺乏导致黄体产生孕酮的最大速率受损。
