Myoclonus, Motor Deficits, Alterations in Emotional Responses and Monoamine Metabolism in ɛ-Sarcoglycan Deficient Mice

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By this author: Yokoi, Fumiaki ;&nbsp; Dang, Mai Tu ;&nbsp; Li, Jianyong ;&nbsp; Li, Yuqing

Authors: Yokoi, Fumiaki; Dang, Mai Tu; Li, Jianyong; Li, Yuqing

Source: The Journal of Biochemistry, Volume 140, Number 1, 15 July 2006 , pp. 141-146(6)

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Abstract:

Mutations of ɛ-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.

Document Type: Research article

DOI: http://dx.doi.org/10.1093/jb/mvj138

Publication date: 2006-07-15