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Journal of Clinical Endocrinology & Metabolism, Vol 71, 984-987, Copyright © 1990 by Endocrine Society

ARTICLES

Steroid secretion by granulosa cells isolated from a woman with 17 alpha-hydroxylase deficiency

C Pariente, J Rabinovici, B Lunenfeld, E Rudak, J Dor, S Mashiach, D Levran, J Blankstein and A Geier
Institute of Endocrinology, Chaim Sheba Medical Center, Tel Hashomer, Israel.

Steroid synthesis in luteal/granulosa cells harvested after follicular aspiration in a patient with 17 alpha-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 alpha-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 alpha-hydroxy progesterone, and androgen levels were minimal. These results provide in vitro evidence of 17 alpha- hydroxylase deficiency in the granulosa cells of this patient.


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