Article
Aggregation and proteasome: the case of elongated polyglutamine aggregation in spinal and bulbar muscular atrophy.
Neurobiology of aging - 1 Jul 2007
Rusmini Paola, Sau Daniela, Crippa Valeria, Palazzolo Isabella, Simonini Francesca, Onesto Elisa, Martini Luciano, Poletti Angelo
Abstract excerpt
Aggregates, a hallmark of most neurodegenerative diseases, may have different properties, and possibly different roles in neurodegeneration. We analysed ubiquitin-proteasome pathway functions during cytoplasmic aggregation in polyglutamine (polyQ) diseases, using a unique model of motor neuron disease, the SpinoBulbar Muscular Atrophy. The disease, which is linked to a polyQ tract elongation in the androgen...
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