Amber L. Southwell, Ph.D. - Publications

2017- Burnett School of Biomedical Sciences-Neuroscience University of Central Florida, Orlando, FL, United States 
Huntington disease, experimental therapeutics, transgenic mice, behavior, stem cells

40 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any innacuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2019 Caron NS, Southwell AL, Brouwers CC, Cengio LD, Xie Y, Black HF, Anderson LM, Ko S, Zhu X, van Deventer SJ, Evers MM, Konstantinova P, Hayden MR. Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease. Nucleic Acids Research. PMID 31745548 DOI: 10.1093/nar/gkz976  1
2018 Southwell AL, Kordasiewicz HB, Langbehn D, Skotte NH, Parsons MP, Villanueva EB, Caron NS, Østergaard ME, Anderson LM, Xie Y, Cengio LD, Findlay-Black H, Doty CN, Fitsimmons B, Swayze EE, et al. Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease. Science Translational Medicine. 10. PMID 30282695 DOI: 10.1126/scitranslmed.aar3959  1
2018 Ehrnhoefer DE, Martin DDO, Schmidt ME, Qiu X, Ladha S, Caron NS, Skotte NH, Nguyen YTN, Vaid K, Southwell AL, Engemann S, Franciosi S, Hayden MR. Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease. Acta Neuropathologica Communications. 6: 16. PMID 29510748 DOI: 10.1186/s40478-018-0518-0  1
2017 Ehrnhoefer DE, Southwell AL, Sivasubramanian M, Qiu X, Villanueva EB, Xie Y, Waltl S, Anderson L, Fazeli A, Casal L, Felczak B, Tsang M, Hayden MR. HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo. Human Molecular Genetics. PMID 29121340 DOI: 10.1093/hmg/ddx394  1
2017 Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Findlay-Black H, Doty CN, Petoukhov E, Iworima D, et al. A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles. Human Molecular Genetics. PMID 28104789 DOI: 10.1093/hmg/ddx021  1
2016 Sanders SS, Parsons MP, Mui KK, Southwell AL, Franciosi S, Cheung D, Waltl S, Raymond LA, Hayden MR. Sudden death due to paralysis and synaptic and behavioral deficits when Hip14/Zdhhc17 is deleted in adult mice. Bmc Biology. 14: 108. PMID 27927242 DOI: 10.1186/s12915-016-0333-7  1
2016 Southwell AL, Smith-Dijak A, Kay C, Sepers M, Villanueva EB, Parsons MP, Xie Y, Anderson L, Felczak B, Waltl S, Ko S, Cheung D, Dal Cengio L, Slama R, Petoukhov E, et al. An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes. Human Molecular Genetics. PMID 27378694 DOI: 10.1093/hmg/ddw212  1
2016 Connolly C, Magnusson-Lind A, Lu G, Wagner PK, Southwell AL, Hayden MR, Björkqvist M, Leavitt BR. Enhanced Immune Response to MMP3 stimulation in Microglia Expressing Mutant Huntingtin. Neuroscience. PMID 27033979 DOI: 10.1016/j.neuroscience.2016.03.031  1
2016 Miniarikova J, Zanella I, Huseinovic A, van der Zon T, Hanemaaijer E, Martier R, Koornneef A, Southwell AL, Hayden MR, van Deventer SJ, Petry H, Konstantinova P. Design, Characterization, and Lead Selection of Therapeutic miRNAs Targeting Huntingtin for Development of Gene Therapy for Huntington's Disease. Molecular Therapy. Nucleic Acids. 5: e297. PMID 27003755 DOI: 10.1038/mtna.2016.7  1
2015 Kay C, Collins JA, Skotte NH, Southwell AL, Warby SC, Caron NS, Doty CN, Nguyen B, Griguoli A, Ross CJ, Squitieri F, Hayden MR. Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry. Molecular Therapy : the Journal of the American Society of Gene Therapy. PMID 26201449 DOI: 10.1038/mt.2015.128  1
2015 Southwell AL, Smith SE, Davis TR, Caron NS, Villanueva EB, Xie Y, Collins JA, Li Ye M, Sturrock A, Leavitt BR, Schrum AG, Hayden MR. Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression. Scientific Reports. 5: 12166. PMID 26174131 DOI: 10.1038/srep12166  1
2015 Mattis VB, Tom C, Akimov S, Saeedian J, Østergaard ME, Southwell AL, Doty CN, Ornelas L, Sahabian A, Lenaeus L, Mandefro B, Sareen D, Arjomand J, Hayden MR, Ross CA, et al. HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity. Human Molecular Genetics. 24: 3257-71. PMID 25740845 DOI: 10.1093/hmg/ddv080  1
2015 Southwell AL, Franciosi S, Villanueva EB, Xie Y, Winter LA, Veeraraghavan J, Jonason A, Felczak B, Zhang W, Kovalik V, Waltl S, Hall G, Pouladi MA, Smith ES, Bowers WJ, et al. Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease. Neurobiology of Disease. 76: 46-56. PMID 25662335 DOI: 10.1016/j.nbd.2015.01.002  1
2015 Østergaard ME, Thomas G, Koller E, Southwell AL, Hayden MR, Seth PP. Biophysical and biological characterization of hairpin and molecular beacon RNase H active antisense oligonucleotides. Acs Chemical Biology. 10: 1227-33. PMID 25654188 DOI: 10.1021/cb500880f  1
2015 Kay C, Collins JA, Skotte NH, Southwell AL, Warby SC, Caron NS, Doty CN, Nguyen B, Griguoli A, Ross CJ, Squitieri F, Hayden MR. Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry Molecular Therapy. DOI: 10.1038/mt.2015.128  1
2014 Southwell AL, Wexler NS. Paul H. Patterson (October 22, 1943 - June 25, 2014). Journal of Huntington's Disease. 3: 221-4. PMID 25300325 DOI: 10.3233/JHD-149006  1
2014 Skotte NH, Southwell AL, Østergaard ME, Carroll JB, Warby SC, Doty CN, Petoukhov E, Vaid K, Kordasiewicz H, Watt AT, Freier SM, Hung G, Seth PP, Bennett CF, Swayze EE, et al. Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients. Plos One. 9: e107434. PMID 25207939 DOI: 10.1371/journal.pone.0107434  1
2014 Southwell AL, Skotte NH, Kordasiewicz HB, Østergaard ME, Watt AT, Carroll JB, Doty CN, Villanueva EB, Petoukhov E, Vaid K, Xie Y, Freier SM, Swayze EE, Seth PP, Bennett CF, et al. In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides. Molecular Therapy : the Journal of the American Society of Gene Therapy. 22: 2093-106. PMID 25101598 DOI: 10.1038/mt.2014.153  1
2014 Kolodziejczyk K, Parsons MP, Southwell AL, Hayden MR, Raymond LA. Striatal synaptic dysfunction and hippocampal plasticity deficits in the Hu97/18 mouse model of Huntington disease. Plos One. 9: e94562. PMID 24728353 DOI: 10.1371/journal.pone.0094562  1
2014 Kay C, Skotte NH, Southwell AL, Hayden MR. Personalized gene silencing therapeutics for Huntington disease. Clinical Genetics. 86: 29-36. PMID 24646433 DOI: 10.1111/cge.12385  1
2014 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, et al. HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response. Proceedings of the National Academy of Sciences of the United States of America. 111: 3032-7. PMID 24516159 DOI: 10.1073/pnas.1314421111  1
2014 Parsons MP, Kang R, Buren C, Dau A, Southwell AL, Doty CN, Sanders SS, Hayden MR, Raymond LA. Bidirectional control of postsynaptic density-95 (PSD-95) clustering by Huntingtin. The Journal of Biological Chemistry. 289: 3518-28. PMID 24347167 DOI: 10.1074/jbc.M113.513945  1
2013 Østergaard ME, Southwell AL, Kordasiewicz H, Watt AT, Skotte NH, Doty CN, Vaid K, Villanueva EB, Swayze EE, Bennett CF, Hayden MR, Seth PP. Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS Nucleic Acids Research. 41: 9634-9650. PMID 23963702 DOI: 10.1093/nar/gkt725  1
2013 Sutton LM, Sanders SS, Butland SL, Singaraja RR, Franciosi S, Southwell AL, Doty CN, Schmidt ME, Mui KK, Kovalik V, Young FB, Zhang W, Hayden MR. Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease. Human Molecular Genetics. 22: 452-65. PMID 23077216 DOI: 10.1093/hmg/dds441  1
2013 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. A fully humanized transgenic mouse model of Huntington disease. Human Molecular Genetics. 22: 18-34. PMID 23001568 DOI: 10.1093/hmg/dds397  1
2012 Graham RK, Deng Y, Pouladi MA, Vaid K, Ehrnhoefer D, Southwell AL, Bissada N, Franciosi S, Hayden MR. Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo. Journal of Huntington's Disease. 1: 243-60. PMID 25063333 DOI: 10.3233/JHD-120038  1
2012 Strong MK, Southwell AL, Yonan JM, Hayden MR, Macgregor GR, Thompson LM, Steward O. Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background. Journal of Huntington's Disease. 1: 221-41. PMID 23833693 DOI: 10.3233/JHD-129005  1
2012 Southwell AL, Skotte NH, Bennett CF, Hayden MR. Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases. Trends in Molecular Medicine. 18: 634-43. PMID 23026741 DOI: 10.1016/j.molmed.2012.09.001  1
2012 Pouladi MA, Stanek LM, Xie Y, Franciosi S, Southwell AL, Deng Y, Butland S, Zhang W, Cheng SH, Shihabuddin LS, Hayden MR. Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice. Human Molecular Genetics. 21: 2219-32. PMID 22328089 DOI: 10.1093/hmg/dds037  1
2011 Carroll JB, Warby SC, Southwell AL, Doty CN, Greenlee S, Skotte N, Hung G, Bennett CF, Freier SM, Hayden MR. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin. Molecular Therapy : the Journal of the American Society of Gene Therapy. 19: 2178-85. PMID 21971427 DOI: 10.1038/mt.2011.201  1
2011 Carroll JB, Southwell AL, Graham RK, Lerch JP, Ehrnhoefer DE, Cao LP, Zhang WN, Deng Y, Bissada N, Henkelman RM, Hayden MR. Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease. Molecular Neurodegeneration. 6: 59. PMID 21854568 DOI: 10.1186/1750-1326-6-59  1
2011 Southwell AL, Patterson PH. Gene therapy in mouse models of huntington disease. The Neuroscientist : a Review Journal Bringing Neurobiology, Neurology and Psychiatry. 17: 153-62. PMID 21489966 DOI: 10.1177/1073858410386236  1
2011 Luong TN, Carlisle HJ, Southwell A, Patterson PH. Assessment of motor balance and coordination in mice using the balance beam. Journal of Visualized Experiments : Jove. PMID 21445033 DOI: 10.3791/2376  1
2011 Southwell AL, Bugg CW, Kaltenbach LS, Dunn D, Butland S, Weiss A, Paganetti P, Lo DC, Patterson PH. Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1. Plos One. 6: e16676. PMID 21304966 DOI: 10.1371/journal.pone.0016676  1
2010 Southwell AL, Patterson PH. Antibody therapy in neurodegenerative disease. Reviews in the Neurosciences. 21: 273-87. PMID 21086760  1
2009 Southwell AL, Ko J, Patterson PH. Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 29: 13589-602. PMID 19864571 DOI: 10.1523/JNEUROSCI.4286-09.2009  1
2008 Southwell AL, Khoshnan A, Dunn DE, Bugg CW, Lo DC, Patterson PH. Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 28: 9013-20. PMID 18768695 DOI: 10.1523/JNEUROSCI.2747-08.2008  1
2005 Chiu CS, Brickley S, Jensen K, Southwell A, Mckinney S, Cull-Candy S, Mody I, Lester HA. GABA transporter deficiency causes tremor, ataxia, nervousness, and increased GABA-induced tonic conductance in cerebellum. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 25: 3234-45. PMID 15788781 DOI: 10.1523/JNEUROSCI.3364-04.2005  1
2003 Zylka MJ, Dong X, Southwell AL, Anderson DJ. Atypical expansion in mice of the sensory neuron-specific Mrg G protein-coupled receptor family. Proceedings of the National Academy of Sciences of the United States of America. 100: 10043-8. PMID 12909716 DOI: 10.1073/pnas.1732949100  1
2001 Lopreato GF, Lu Y, Southwell A, Atkinson NS, Hillis DM, Wilcox TP, Zakon HH. Evolution and divergence of sodium channel genes in vertebrates. Proceedings of the National Academy of Sciences of the United States of America. 98: 7588-92. PMID 11416226 DOI: 10.1073/pnas.131171798  1
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