The fetal testis produces testosterone, the hormone
that induces male sexual differentiation.
Testosterone is taken up by cells of the target
tissues (wolffian ducts and urogenital sinus)
(1). In the urogenital sinus, testosterone is converted
into dihydrotestosterone (DHT) by the
enzyme 5!-reductase. Both testosterone and
dihydrotestosterone bind to an intracellular receptor
(androgen receptor). The activated hormone–
receptor complex (TR* or DR*) acts as a
transcription factor for genes that regulate the
differentiation of thewolffian ducts and the urogenital
sinus. Thus, normal male fetal development
is dependent on normal biosynthesis of
testosterone and normal receptors. Androgen
receptor mutations lead to disorders of sexual
development (2) with X-chromosomal inherited
complete or incomplete androgen resistance
(testicular feminization, TFM).
Sunday, April 12, 2009
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