The analysis of participation in the ubiquitin proteasome system for understanding the mechanisms of neurodegenerative diseases.

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We have established motor neuron-specific proteasome deficient mice by crossbreeding of flexed mice for Rpt3, a subunit of 26S proteasome with VAChT-Cre. Fast mice that displays motor neuron specific Cre expression. The motor neuron specific Rpt3 deficient mice showed tremor starting from 8 weeks of age and slowly progressive motor dysfunction from 12 weeks of age. Pathological findings revealed increased expression of the gene products of familial ALS such as FUS and TDP43, the latter forming cytoplasmic inclusion bodies. These results suggest that motor neuron-specific Rpt3 deficient mice represent a model for sporadic ALS.