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Steroid Secretion by Granulosa Cells Isolated from a Woman with 17a-Hydroxylase Deficiency

Institute of Endocrinology (C.P., B.L., A.G.) Tel Hashomer, Israel
The Department of Obstetrics and Gynecology (J.R., E.R., J.D., S.M., D.L., J.B.), the Chaim Sheba Medical Center Tel Hashomer, Israel

Address requests for reprints to: Clara Pariente, MSc, Institute of Endocrinology, Chaim Sheba Medical Center, Tel Hashomer, 52621 Israel.

Steroid synthesis in luteal/granulosa cells harvested after follicular aspiration in a patient with 17-hydroxylase deficiency was investigated. Follicular stimulation with purified FSH after the suppression of ACTH and gonadotropin secretion by corticoid analogs and superactive GnRH agonist permitted ovum pick-up and the study of steroid synthesis in the obtained granulosa cells. Progesterone synthesis was elevated while 17-hydroxy progesterone, testosterone, androstenedione, and estrogen production was minimal. Aromatase activity was retained in these cells, as demonstrated by the 100-fold increase in estrogen levels after the addition of androgens. Progesterone levels in follicular fluids were in the normal range, while estrogen, 17-hydroxy progesterone, and androgen levels were minimal. These results provide in vitro evidence of 17-hydroxylase deficiency in the granulosa cells of this patient.

Received November 13, 1989.

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