Assessment of motor balance and coordination in mice using the balance beam TN Luong, HJ Carlisle, A Southwell, PH Patterson JoVE (Journal of Visualized Experiments), e2376, 2011 | 433 | 2011 |
Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene/allele-specific silencing of mutant huntingtin JB Carroll, SC Warby, AL Southwell, CN Doty, S Greenlee, N Skotte, ... Molecular Therapy 19 (12), 2178-2185, 2011 | 315 | 2011 |
Atypical expansion in mice of the sensory neuron-specific Mrg G protein-coupled receptor family MJ Zylka, X Dong, AL Southwell, DJ Anderson Proceedings of the National Academy of Sciences 100 (17), 10043-10048, 2003 | 284 | 2003 |
Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease AL Southwell, J Ko, PH Patterson Journal of Neuroscience 29 (43), 13589-13602, 2009 | 271 | 2009 |
GABA transporter deficiency causes tremor, ataxia, nervousness, and increased GABA-induced tonic conductance in cerebellum CS Chiu, S Brickley, K Jensen, A Southwell, S Mckinney, S Cull-Candy, ... Journal of Neuroscience 25 (12), 3234-3245, 2005 | 259 | 2005 |
Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS ME Østergaard, AL Southwell, H Kordasiewicz, AT Watt, NH Skotte, ... Nucleic acids research 41 (21), 9634-9650, 2013 | 179 | 2013 |
Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice MA Pouladi, LM Stanek, Y Xie, S Franciosi, AL Southwell, Y Deng, ... Human molecular genetics 21 (10), 2219-2232, 2012 | 177 | 2012 |
In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides AL Southwell, NH Skotte, HB Kordasiewicz, ME Østergaard, AT Watt, ... Molecular Therapy 22 (12), 2093-2106, 2014 | 160 | 2014 |
Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases AL Southwell, NH Skotte, CF Bennett, MR Hayden Trends in molecular medicine 18 (11), 634-643, 2012 | 157 | 2012 |
Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients NH Skotte, AL Southwell, ME Østergaard, JB Carroll, SC Warby, CN Doty, ... PloS one 9 (9), e107434, 2014 | 153 | 2014 |
Design, characterization, and lead selection of therapeutic miRNAs targeting huntingtin for development of gene therapy for Huntington's disease J Miniarikova, I Zanella, A Huseinovic, T van der Zon, E Hanemaaijer, ... Molecular Therapy-Nucleic Acids 5, 2016 | 149 | 2016 |
Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity AL Southwell, A Khoshnan, DE Dunn, CW Bugg, DC Lo, PH Patterson Journal of Neuroscience 28 (36), 9013-9020, 2008 | 129 | 2008 |
HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity VB Mattis, C Tom, S Akimov, J Saeedian, ME Østergaard, AL Southwell, ... Human molecular genetics 24 (11), 3257-3271, 2015 | 124 | 2015 |
A fully humanized transgenic mouse model of Huntington disease AL Southwell, SC Warby, JB Carroll, CN Doty, NH Skotte, W Zhang, ... Human molecular genetics 22 (1), 18-34, 2013 | 123 | 2013 |
Huntingtin suppression restores cognitive function in a mouse model of Huntington’s disease AL Southwell, HB Kordasiewicz, D Langbehn, NH Skotte, MP Parsons, ... Science translational medicine 10 (461), eaar3959, 2018 | 120 | 2018 |
Evolution and divergence of sodium channel genes in vertebrates GF Lopreato, Y Lu, A Southwell, NS Atkinson, DM Hillis, TP Wilcox, ... Proceedings of the National Academy of Sciences 98 (13), 7588-7592, 2001 | 119 | 2001 |
Huntingtin haplotypes provide prioritized target panels for allele-specific silencing in Huntington disease patients of European ancestry C Kay, JA Collins, NH Skotte, AL Southwell, SC Warby, NS Caron, ... Molecular Therapy 23 (11), 1759-1771, 2015 | 115 | 2015 |
An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes AL Southwell, A Smith-Dijak, C Kay, M Sepers, EB Villanueva, ... Human molecular genetics 25 (17), 3654-3675, 2016 | 110 | 2016 |
Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression AL Southwell, SEP Smith, TR Davis, NS Caron, EB Villanueva, Y Xie, ... Scientific reports 5 (1), 12166, 2015 | 110 | 2015 |
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response B Rotblat, AL Southwell, DE Ehrnhoefer, NH Skotte, M Metzler, ... Proceedings of the National Academy of Sciences 111 (8), 3032-3037, 2014 | 108 | 2014 |