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Amber L Southwell
Amber L Southwell
Burnett School of Biomedical Sciences, University of Central Florida
Adresse e-mail validée de ucf.edu - Page d'accueil
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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
4092011
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
3112011
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
2772003
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
2652009
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
2532005
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
1742013
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
1722012
Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases
AL Southwell, NH Skotte, CF Bennett, MR Hayden
Trends in molecular medicine 18 (11), 634-643, 2012
1542012
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
1492014
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
1492014
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
1392016
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
1262008
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
1242013
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
1192015
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
1182001
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
1112015
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
1082018
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
1082015
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
1042016
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
1032014
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