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Front Neuroendocrinol. 2014 Oct;35(4):405-19. doi: 10.1016/j.yfrne.2013.12.004. Epub 2014 Jan 2.

Mouse model systems to study sex chromosome genes and behavior: relevance to humans.

Author information

1
Department of Biochemistry and Molecular Genetics and Program in Neuroscience, University of Virginia School of Medicine, Charlottesville, VA 22908, United States.
2
Department of Biochemistry and Molecular Genetics and Program in Neuroscience, University of Virginia School of Medicine, Charlottesville, VA 22908, United States. Electronic address: Rissman@virginia.edu.

Abstract

Sex chromosome genes directly influence sex differences in behavior. The discovery of the Sry gene on the Y chromosome (Gubbay et al., 1990; Koopman et al., 1990) substantiated the sex chromosome mechanistic link to sex differences. Moreover, the pronounced connection between X chromosome gene mutations and mental illness produces a strong sex bias in these diseases. Yet, the dominant explanation for sex differences continues to be the gonadal hormones. Here we review progress made on behavioral differences in mouse models that uncouple sex chromosome complement from gonadal sex. We conclude that many social and cognitive behaviors are modified by sex chromosome complement, and discuss the implications for human research. Future directions need to include identification of the genes involved and interactions with these genes and gonadal hormones.

KEYWORDS:

Behavior; Four core genotypes; Klinefelter syndrome; Sex differences; Sexual differentiation; Turner syndrome

PMID:
24388960
PMCID:
PMC4079771
DOI:
10.1016/j.yfrne.2013.12.004
[Indexed for MEDLINE]
Free PMC Article

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