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Spinocerebellar ataxia 2 (SCA2).
Lastres-Becker I, Rüb U, Auburger G. Lastres-Becker I, et al. Cerebellum. 2008;7(2):115-24. doi: 10.1007/s12311-008-0019-y. Cerebellum. 2008. PMID: 18418684 Review.
Although these disorders differ clinically and in the affected neuron populations, it is not understood how the different polyglutamine proteins mediate such tissue specificity. The neuronal intranuclear inclusion bodies described in other polyglutamin …
Although these disorders differ clinically and in the affected neuron populations, it is not understood how the different polyglutamine prot …
Oxidative stress in Huntington's disease.
Browne SE, Ferrante RJ, Beal MF. Browne SE, et al. Brain Pathol. 1999 Jan;9(1):147-63. doi: 10.1111/j.1750-3639.1999.tb00216.x. Brain Pathol. 1999. PMID: 9989457 Free PMC article. Review.
Importantly, the recent development of transgenic mouse models of HD led to the identification of neuronal intranuclear inclusion bodies in affected brain regions in both mouse models and in HD brain, consisting of protein aggregates containing fragmen …
Importantly, the recent development of transgenic mouse models of HD led to the identification of neuronal intranuclear inc
Pathogenesis of neurodegenerative diseases associated with expanded glutamine repeats: new answers, new questions.
Ross CA, Margolis RL, Becher MW, Wood JD, Engelender S, Cooper JK, Sharp AH. Ross CA, et al. Prog Brain Res. 1998;117:397-419. doi: 10.1016/s0079-6123(08)64029-7. Prog Brain Res. 1998. PMID: 9932422 Free article. Review.
Disease age of onset, and in some cases severity, is correlated with repeat length. These and other observations have led to the hypothesis that CAG expansion causes disease by a toxic gain-of-function of the encoded stretch of polyglutamine residues. ...Recent findings ha …
Disease age of onset, and in some cases severity, is correlated with repeat length. These and other observations have led to the hypo …