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Review
. 2010 Apr;21(2):194-200.
doi: 10.1016/j.semcdb.2009.09.017. Epub 2009 Oct 6.

X inactivation and disease

Ruben Agrelo  1 , Anton Wutz
Affiliations
Review

X inactivation and disease

Ruben Agrelo et al. Semin Cell Dev Biol. 2010 Apr.

Abstract

X inactivation is the mechanism by which mammals adjust the X-linked gene dosage between the sexes. The dosage difference between XX females and XY males is functionally equalized by silencing one of the two X chromosomes in female cells. This dosage-compensation mechanism is based on the long functional Xist RNA. Here, we review our understanding of dosage compensation and Xist function in the context of disease.

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