Ubiquitin-positive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation.

@article{Wenzel2010UbiquitinpositiveII,
  title={Ubiquitin-positive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation.},
  author={H. J{\"u}rgen Wenzel and Michael R Hunsaker and Claudia M. Greco and Rob Willemsen and Robert F. Berman},
  journal={Brain research},
  year={2010},
  volume={1318},
  pages={155-66}
}
Fragile X-associated tremor/ataxia syndrome (FXTAS) is an adult-onset neurodegenerative disorder caused by CGG trinucleotide repeat expansions in the fragile X mental retardation 1 (FMR1) gene. The neuropathological hallmark of the disease is the presence of ubiquitin-positive intranuclear inclusions in neurons and in astrocytes. Ubiquitin-positive intranuclear inclusions have also been found in the neurons of transgenic mice model carrying an expanded CGG((98)) trinucleotide repeat of human… CONTINUE READING
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