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Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction.
Ghosh R, Wood-Kaczmar A, Dobson L, Smith EJ, Sirinathsinghji EC, Kriston-Vizi J, Hargreaves IP, Heaton R, Herrmann F, Abramov AY, Lam AJ, Heales SJ, Ketteler R, Bates GP, Andre R, Tabrizi SJ. Ghosh R, et al. Among authors: smith ej. FASEB J. 2020 Jun;34(6):8139-8154. doi: 10.1096/fj.201902277RR. Epub 2020 Apr 23. FASEB J. 2020. PMID: 32329133 Free PMC article.
Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice.
Bondulich MK, Jolinon N, Osborne GF, Smith EJ, Rattray I, Neueder A, Sathasivam K, Ahmed M, Ali N, Benjamin AC, Chang X, Dick JRT, Ellis M, Franklin SA, Goodwin D, Inuabasi L, Lazell H, Lehar A, Richard-Londt A, Rosinski J, Smith DL, Wood T, Tabrizi SJ, Brandner S, Greensmith L, Howland D, Munoz-Sanjuan I, Lee SJ, Bates GP. Bondulich MK, et al. Among authors: smith dl, smith ej. Sci Rep. 2017 Oct 27;7(1):14275. doi: 10.1038/s41598-017-14290-3. Sci Rep. 2017. PMID: 29079832 Free PMC article.
Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington'S Disease Model.
Landles C, Milton RE, Ali N, Flomen R, Flower M, Schindler F, Gomez-Paredes C, Bondulich MK, Osborne GF, Goodwin D, Salsbury G, Benn CL, Sathasivam K, Smith EJ, Tabrizi SJ, Wanker EE, Bates GP. Landles C, et al. Among authors: smith ej. Brain Commun. 2020 Aug 3;2(2):fcaa066. doi: 10.1093/braincomms/fcaa066. eCollection 2020. Brain Commun. 2020. PMID: 32954323 Free PMC article.
567 results