[Genetics and endocrinology of male sex differentiation: application to molecular study of male pseudohermaphroditism].
The various processes involved in sexual differentiation have been considerably clarified over the last few years through advances in biochemistry and molecular genetics. The cloning of the gene responsible for testicular determination SRY, of the anti-Müllerian hormone and anti-Müllerian hormone receptor genes, of the several steroidogenic enzymes genes, of the 5 alpha-reductase type 2 gene and of the androgen receptor gene has permitted to elucidate the molecular defects causing abnormal sexual differentiation. These data have brought a substantial impact on the understanding of human male sexual differentiation and its main disorders.
Sultan
C
C
INSERM U439, Hôpital Lapeyronie, Montpellier, France.
Lumbroso
S
S
Poujol
N
N
Boudon
C
C
Georget
V
V
Térouanne
B
B
Belon
C
C
Lobaccaro
J M
JM
D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase / Q000172:deficiency; D000312:Adrenal Hyperplasia, Congenital / Q000201:enzymology; D000728:Androgens / Q000378:metabolism; D012734:Disorders of Sex Development / Q000235:genetics* / Q000378:metabolism*; D006059:Gonadal Dysgenesis / Q000235:genetics; D006801:Humans; D008297:Male; D012732:Sex Determination Analysis; D012733:Sex Differentiation; D013737:Testis / Q000002:abnormalities
D000728:Androgens; D015096:3-Hydroxysteroid Dehydrogenases; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase
Génétique et endocrinologie de la différenciation sexuelle masculine: application à l'étude moléculaire des pseudohermaphrodismes masculins.