Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

By H. Jürgen Wenzel, Karl D. Murray, Saif N. Haify, Michael R. Hunsaker, Jared J. Schwartzer, Kyoungmi Kim, Albert R. La Spada, Bryce L. Sopher, Paul J. Hagerman, Christopher Raske, Lies-Anne W.F.M. Severijnen, Rob Willemsen, Renate K. Hukema and Robert F. Berman On Feb 26, 2019

The fragile X premutation is a CGG trinucleotide repeat expansion between 55 and 200 repeats in the 5′-untranslated region of the fragile X mental retardation 1 (FMR1) gene. Human carriers of the premutation alle…

neuropathology

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