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Affected individuals have sex chromosomes showing male-female mosaicism (where one individual possesses both the male XY and female XX chromosome pairs).

Most often, but not always, the chromosome complement is 46, XX, and in every such individual there also exists evidence of Y chromosomal material on one of the autosomes (any of the 22 pairs of chromosomes other than the sex chromosomes).

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The ovarian and testicular tissue may be separate, or the two may be combined in what is called an ovotestis.

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