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Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice.
Pouladi MA, Stanek LM, Xie Y, Franciosi S, Southwell AL, Deng Y, Butland S, Zhang W, Cheng SH, Shihabuddin LS, Hayden MR. Pouladi MA, et al. Among authors: southwell al. Hum Mol Genet. 2012 May 15;21(10):2219-32. doi: 10.1093/hmg/dds037. Epub 2012 Feb 9. Hum Mol Genet. 2012. PMID: 22328089
A fully humanized transgenic mouse model of Huntington disease.
Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR. Southwell AL, et al. Hum Mol Genet. 2013 Jan 1;22(1):18-34. doi: 10.1093/hmg/dds397. Epub 2012 Sep 21. Hum Mol Genet. 2013. PMID: 23001568 Free PMC article.
Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS.
Østergaard ME, Southwell AL, Kordasiewicz H, Watt AT, Skotte NH, Doty CN, Vaid K, Villanueva EB, Swayze EE, Bennett CF, Hayden MR, Seth PP. Østergaard ME, et al. Among authors: southwell al. Nucleic Acids Res. 2013 Nov;41(21):9634-50. doi: 10.1093/nar/gkt725. Epub 2013 Aug 19. Nucleic Acids Res. 2013. PMID: 23963702 Free PMC article.
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response.
Rotblat B, Southwell AL, Ehrnhoefer DE, Skotte NH, Metzler M, Franciosi S, Leprivier G, Somasekharan SP, Barokas A, Deng Y, Tang T, Mathers J, Cetinbas N, Daugaard M, Kwok B, Li L, Carnie CJ, Fink D, Nitsch R, Galpin JD, Ahern CA, Melino G, Penninger JM, Hayden MR, Sorensen PH. Rotblat B, et al. Among authors: southwell al. Proc Natl Acad Sci U S A. 2014 Feb 25;111(8):3032-7. doi: 10.1073/pnas.1314421111. Epub 2014 Feb 10. Proc Natl Acad Sci U S A. 2014. PMID: 24516159 Free PMC article.
54 results