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Human Molecular Genetics Advance Access published online on May 26, 2004
Human Molecular Genetics, doi:10.1093/hmg/ddh161
© 2004 by Oxford University Press
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Article

Six5 is required for spermatogenic cell survival and spermiogenesis

Partha S. Sarkar 1, Sharan Paul 1, Jennifer Han 1, Sita Reddy 1*

1 Institute for Genetic Medicine, Room 240, University of Southern California, Keck School of Medicine, 2250 Alcazar Street, Los Angeles, CA 90033

* To whom correspondence should be addressed. E-mail: sitaredd{at}usc.edu.


  Abstract

Myotonic dystrophy 1 (DM1) is a multi-system disorder characterized by endocrine defects that include testicular and tubular atrophy, oligospermia, leydig cell hyper proliferation and increased FSH levels. DM1 results from a CTG expansion that causes transcriptional silencing of the flanking SIX5 allele. Loss of Six5 results in male sterility and a progressive decrease in testicular mass with age. We demonstrate a strict requirement of Six5 both for spermatogenic cell survival and spermiogenesis. Leydig cell hyper proliferation and increased intra testicular testosterone levels are observed in Six5-/- mice. Although increased FSH levels are observed in Six5+/- and Six5-/- mice, serum testosterone levels and intra testicular inhibin alpha and inhibin beta B levels are not altered in Six5 mutant animals when compared to controls. Significantly, steady state c-Kit levels are reduced in Six5-/- testis. Thus decreased c-Kit levels could contribute to the elevated spermatogenic cell apoptosis and leydig cell hyper proliferation in Six5-/- mice. The results support the hypothesis that reduced SIX5 levels contribute to the male reproductive defects in DM1.


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