Comprehensive study on dystonia pathology

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In the mammalian striatum, there exist two functional subdivisions termed as striosome and matrix compartments. We have proposed a hypothesis that an imbalanced activities between the striosome and matrix compartments may cause movement disorders that include dystonia. During the last 3 years, we found a selective loss of dopamine D1 receptor D1R and Golf expression in the striosomes in a transgenic mouse model with dystonia, and suggest that a loss of D1Rmediated signaling in the striosomes may cause dystonia symptoms. Moreover, our pathological study on the autopsied brains showed the defects in the neuropeptide NPY system in the striatum of the patients with DYT3 dystonia. Since NPY has a protective role against glutamate receptor-mediated neurotoxicity and promotes striatal neurogenesis, this finding suggests that defects in the NPY system may underlie the genesis of progressive striatal generation that primarily occurs in the striosome compartment.