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Reduced bioavailable manganese causes striatal urea cycle pathology in Huntington's disease mouse model.
Bichell TJV, Wegrzynowicz M, Tipps KG, Bradley EM, Uhouse MA, Bryan M, Horning K, Fisher N, Dudek K, Halbesma T, Umashanker P, Stubbs AD, Holt HK, Kwakye GF, Tidball AM, Colbran RJ, Aschner M, Neely MD, Di Pardo A, Maglione V, Osmand A, Bowman AB. Bichell TJV, et al. Biochim Biophys Acta Mol Basis Dis. 2017 Jun;1863(6):1596-1604. doi: 10.1016/j.bbadis.2017.02.013. Epub 2017 Feb 16. Biochim Biophys Acta Mol Basis Dis. 2017. PMID: 28213125 Free PMC article.
Further molecular characterisation of the OVT73 transgenic sheep model of Huntington's disease identifies cortical aggregates.
Huntington's Disease Sheep Collaborative Research Group; Reid SJ, Patassini S, Handley RR, Rudiger SR, McLaughlan CJ, Osmand A, Jacobsen JC, Morton AJ, Weiss A, Waldvogel HJ, MacDonald ME, Gusella JF, Bawden CS, Faull RL, Snell RG. Huntington's Disease Sheep Collaborative Research Group, et al. J Huntingtons Dis. 2013;2(3):279-95. doi: 10.3233/JHD-130067. J Huntingtons Dis. 2013. PMID: 25062676
Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease.
Lu XH, Mattis VB, Wang N, Al-Ramahi I, van den Berg N, Fratantoni SA, Waldvogel H, Greiner E, Osmand A, Elzein K, Xiao J, Dijkstra S, de Pril R, Vinters HV, Faull R, Signer E, Kwak S, Marugan JJ, Botas J, Fischer DF, Svendsen CN, Munoz-Sanjuan I, Yang XW. Lu XH, et al. Sci Transl Med. 2014 Dec 24;6(268):268ra178. doi: 10.1126/scitranslmed.3010523. Sci Transl Med. 2014. PMID: 25540325
47 results