Steroid secretion by granulosa cells isolated from a woman with 17 alpha-hydroxylase deficiency.
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.
Pariente
C
C
Institute of Endocrinology, Chaim Sheba Medical Center, Tel Hashomer, Israel.
Rabinovici
J
J
Lunenfeld
B
B
Rudak
E
E
Dor
J
J
Mashiach
S
S
Levran
D
D
Blankstein
J
J
Geier
A
A
D000312:Adrenal Hyperplasia, Congenital; D000328:Adult; D000735:Androstenedione / Q000378:metabolism; D001826:Body Fluids / Q000378:metabolism; D002478:Cells, Cultured; D004967:Estrogens / Q000378:metabolism*; D005260:Female; D005498:Follicular Phase / Q000502:physiology; D006107:Granulosa Cells / Q000378:metabolism*; D006801:Humans; D006908:Hydroxyprogesterones / Q000378:metabolism; D008183:Luteal Phase / Q000502:physiology; D011374:Progesterone / Q000378:metabolism*; D013254:Steroid 17-alpha-Hydroxylase / Q000378:metabolism; D013250:Steroid Hydroxylases / Q000172:deficiency*; D013739:Testosterone / Q000378:metabolism*
D004967:Estrogens; D006908:Hydroxyprogesterones; D013739:Testosterone; D000735:Androstenedione; D011374:Progesterone; D013250:Steroid Hydroxylases; D013254:Steroid 17-alpha-Hydroxylase