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In rare cases, 46,XX gonads may differentiate into testes, a condition known as 46,XX testicular disorders of sex development (DSD), or give rise to the coexistence of ovarian and testicular tissue, a condition known as 46,XX ovotesticular DSD. Testicular tissue differentiation may be due to the translocation of SRY to the X chromosome or an autosome. In the absence of SRY, overexpression of other pro-testis genes, e.g. SOX family genes, or failure of pro-ovarian\/anti-testis genes, such as WNT4 and RSPO1, may underlie the development of testicular tissue. Recent experimental and clinical evidence giving insight into SRY-negative 46,XX testicular or ovotesticular DSD is discussed.<\/jats:p>","DOI":"10.1159\/000445088","type":"journal-article","created":{"date-parts":[[2016,4,8]],"date-time":"2016-04-08T11:33:30Z","timestamp":1460115210000},"page":"1-11","source":"Crossref","is-referenced-by-count":69,"title":["Disorders of Sex Development with Testicular Differentiation in SRY-Negative 46,XX Individuals: Clinical and Genetic Aspects"],"prefix":"10.1159","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6291-1518","authenticated-orcid":false,"given":"Romina P.","family":"Grinspon","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1100-3843","authenticated-orcid":false,"given":"Rodolfo 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