Aromatase deficiency- Aromatase is an enzyme that normally converts male hormones to female hormones.Excessive aromatase activity leads to the overproduction of female hormone estrogen while its deficiency can result in 46, XX intersex, where enough estrogen is not produced. The condition used to be called male pseudohermaphroditism.
These occur due to mutations in the autosomal genes DHCR7 (which codes for 7-dehydrocholesterol reductase) or it could be due to deletions in the autosomal gene SOX-9 In some cases of 46XY there is a loss of SRY genes located on the Y chromosomes .
Mutation in the gene DAX 1 is also associated with XY females Swyer syndrome is a type of hypogonadism.
Here the functional gonads,the ovaries, are absent but the person resembles a normal female and has a karyotype 46, XY.
And now at last, after all these years, the ivory towers of these self appointed gods and kings have collapsed and their wrongdoings have been disclosed to all the world.
With the CASTER SEMENYA case they have finally been forced to admit that they do not know how to decide the gender of a human being !!