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Biosynthesis of Steroids in Various Tissues Related to Feminizing Syndromes

( Worcester Foundation for Experimental Biology, Shrewsbury, Massachusetts; Institute of Hormone Biology, Syntex Research Center, Palo Alto, California; The New York Medical College, New York, New York; and The Mount Sinai Hospital, New York, New York)

Recently we had an opportunity to study the biochemistry of 2 clinical conditions leading to feminization: one due to an adrenal tumor and the other to abnormal testicular tissue. In the 1st instance, the biosynthesis of estrogens from pregnenolone and progesterone in an adrenal tumor was demonstrated for the 1st time. In the 2nd study, estrogen biosynthesis was demonstrated by homogenates of these abnormal testes, and it was shown that these patients have normal levels of testosterone in the plasma even though no true masculinization is observed. A hypothesis accounting for this abnormality is suggested.