Campomelic dysplasia and autosomal sex reversal caused by mutations in an SRY-related gene.

Foster, J W; Dominguez-Steglich, M A; Guioli, S; Kwok, C; Weller, P A; Stevanović, M; Weissenbach, J; Mansour, S et al. · Nature · 1994

case_report · Level V

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Abstract

Induction of testis development in mammals requires the presence of the Y-chromosome gene SRY. This gene must exert its effect by interacting with other genes in the sex-determination pathway. Cloning of a translocation chromosome breakpoint from a sex-reversed patient with campomelic dysplasia, followed by mutation analysis of an adjacent gene, indicates that SOX9, an SRY-related gene, is involved in both bone formation and control of testis development.

Medical subject headings