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The activities in chorion and placenta were very similar with respect to subcellular localization, specific activity, substrate specificity, nucleotide specificity, and steroid binding properties. This group of investigators has also shown that both chorion and amnion contain the aromatase complex ( Gibb and Lavoie, 1981 ). Thus, it is apparent that the fetal membranes have the full potential to synthesize estrogens and progesterone from DHEAS and P 5 S, a not surprising conclusion in view of the common trophoblastic origin of the placenta and chorion laeve. Amniotic fluid contains an abundant supply of steroidal sulfates that can serve as substrates for these biosynthetic enzymes, including DHEAS, 16-OH-DHEAS, pregnenolone, and 16 α -OH-P 5 S ( Schindler and Siiteri, 1968 ). Furthermore, the pattern of steroids found in amniotic fluid derived from fetal urine appears to closely reflect fetal adrenal secretory activity ( Sippell et al ., 1981 ). 21OHD and 11OHD impair steroidogenesis in the adrenal glands only [1,2,3, 6, 17]. In contrast, severe form of 3βHSD2D causes defects of steroidogenesis in both adrenal glands and gonads [2, 3, 15]. Figure 2 illustrates the pathophysiology of 3βHSD2D. Inhaltsverzeichnis.
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