Amber L Southwell
Amber L Southwell
Burnett School of Biomedical Sciences, University of Central Florida
Verified email at ucf.edu - Homepage
TitleCited byYear
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
1962011
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
1962009
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
1942005
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
1942003
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
1512011
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
1212013
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
1062012
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
1012001
Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases
AL Southwell, NH Skotte, CF Bennett, MR Hayden
Trends in molecular medicine 18 (11), 634-643, 2012
932012
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
922014
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
912008
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
802014
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
672015
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, 2012
602012
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
552015
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
552014
Personalized gene silencing therapeutics for Huntington disease
C Kay, NH Skotte, AL Southwell, MR Hayden
Clinical genetics 86 (1), 29-36, 2014
512014
Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease
AL Southwell, S Franciosi, EB Villanueva, Y Xie, LA Winter, ...
Neurobiology of disease 76, 46-56, 2015
472015
Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease
LM Sutton, SS Sanders, SL Butland, RR Singaraja, S Franciosi, ...
Human molecular genetics 22 (3), 452-465, 2012
472012
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, 12166, 2015
442015
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Articles 1–20