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inclusion protein neuron sam 68 level cgg aggregates brain mouse hagerman aggregate precgg figure patient premutation syndrome fxtas patients intranuclear intranuclear inclusions model genet carrier tremor tassone fmr 1 mrna culture antibody fmr 1 gene cos 7 cells expression analysis control precgg neurons mouse model cgg mice /ataxia mbnl 1 study x syndrome result gland number transfection pm carriers erasmus university rotterdam ubiquitin-positive formation hippocampal expanded-repeat ubiquitin-positive intranuclear inclusions 60 cgg corticosterone wt mice corticosterone levels signi disease littermate alternative dendritic hnrnp-g willemsen presence tautomycin sam 68 protein 21 div genetic plasmid expansion regulation premutation carriers length 2010 european symptom hippocampal neurons expanded-repeat mice wt littermates allele nuclei amygdala cgg rna aggregates transfected
4 Most Recent Publications
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Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients
(Article)
Sellier, C. Rau, F. Hagerman, P.J. Charlet-Berguerand, N. Liu, Y.X. Tassone, F. Hukema, R.K. Gattoni, R. Schneider, A. Richard, S. Willemsen, R. Elliott, D.J. |
2010-04-01
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Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degeneration
(Article)
Chen, Y. Tassone, F. Berman, R.F. Hagerman, P.J. Willemsen, R. Pessah, I.N. |
2009-10-21
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Altered hypothalamus-pituitary-adrenal gland axis regulation in the expanded CGG-repeat mouse model for fragile X-associated tremor/ataxia syndrome
(Article)
Brouwer, J. Severijnen, E.A. Jong, F.H. de Hessl, D. Hagerman, P.J. Oostra, B.A. Willemsen, R. |
2008-07-01
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The FMR1 CGG repeat mouse displays ubiquitin-positive intranuclear neuronal inclusions; implications for the cerebellar tremor/ataxia syndrome
(Article)
Willemsen, R. Unen, L. van Severijnen, L.A. Nieuwenhuizen, I.M. Schrier, M. Tassone, F. Oostra, B.A. Hoogeveen, A.T. Mientjes, E.J. Hagerman, P.J. Hoogeveen-Westerveld, M. Reis, S. Holstege, J. |
2003-01-01
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