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Journal of Biochemistry Advance Access originally published online on June 30, 2006
Journal of Biochemistry 2006 140(1):141-146; doi:10.1093/jb/mvj138
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© 2006 The Japanese Biochemical Society.

Regular Paper

Myoclonus, Motor Deficits, Alterations in Emotional Responses and Monoamine Metabolism in {varepsilon}-Sarcoglycan Deficient Mice

Fumiaki Yokoi1, Mai Tu Dang1, Jianyong Li2 and Yuqing Li1,*

1 Department of Molecular and Integrative Physiology, Beckman Institute for Advanced Science and Technology; and 2 Department of Pathobiology, University of Illinois at Urbana-Champaign, 405 N. Mathews Ave., Urbana, Illinois 61801, USA

* To whom correspondence should be addressed. 3347 Beckman Institute, 405 N. Mathews Ave., Urbana, Illinois 61801, USA. Tel: +1-217-244-1614, Fax: +1-217-244-1726, E-mail: y-li4{at}uiuc.edu

Mutations of {varepsilon}-sarcoglycan gene (SGCE) have been implicated in myoclonus-dystonia (M-D), a movement disorder. To determine the pathophysiology of M-D, we produced Sgce knockout mice and found that the knockout mice exhibited myoclonus, motor impairments, hyperactivity, anxiety, depression, significantly higher levels of striatal dopamine and its metabolites, and an inverse correlation between the dopamine and serotonin metabolites. The results suggest that the diverse symptoms associated with M-D are indeed resulted from a single SGCE gene mutation that leads to alterations of dopaminergic and serotonergic systems. Therefore, antipsychotic agents and serotonin reuptake inhibitors may offer potential benefits for M-D patients.


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