Complete testicular feminization syndrome is characterized by female phenotype, in spite of the normal male karyotype 46, XY. There is a congenital insensitivity to androgens, transmitted by means of a maternal X-linked recessive gene responsible
for
the androgen intracellular receptor. Therefore, androgen induction of Wolffian duct development does not occur. However, antimullerian hormone activity is present, and the individual does not have mullerian development.
|