Biological Aging and the Cellular Pathogenesis of Huntington’s Disease

por Machiela, Emily, Southwell, Amber L.
Publicado en J Huntingtons Dis (2020)

Gene Therapy in Mouse Models of Huntington Disease

por Southwell, Amber L., Patterson, Paul H.
Publicado 2011

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

por Southwell, Amber L., Ko, Jan, Patterson, Paul H.
Publicado 2009

Childhood cat bites relate to increased adulthood severity of schizotypy, psychotic-like experiences, and social anhedonia in a transdiagnostic psychiatric sample

por Bedwell, Jeffrey S., Spencer, Christopher C., Southwell, Amber L.
Publicado en Psychopathology (2020)

Atypical expansion in mice of the sensory neuron-specific Mrg G protein-coupled receptor family

por Zylka, Mark J., Dong, Xinzhong, Southwell, Amber L., Anderson, David J.
Publicado 2003

Striatal Synaptic Dysfunction and Hippocampal Plasticity Deficits in the Hu97/18 Mouse Model of Huntington Disease

por Kolodziejczyk, Karolina, Parsons, Matthew P., Southwell, Amber L., Hayden, Michael R., Raymond, Lynn A.
Publicado 2014

Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity

por Southwell, Amber L., Khoshnan, Ali, Dunn, Denise E., Bugg, Charles W., Lo, Donald C., Patterson, Paul H.
Publicado 2008

Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background

por Strong, Melissa K., Southwell, Amber L., Yonan, Jennifer M., Hayden, Michael R., MacGregor, Grant R., Thompson, Leslie M., Steward, Oswald
Publicado 2012

Perturbation with Intrabodies Reveals That Calpain Cleavage Is Required for Degradation of Huntingtin Exon 1

por Southwell, Amber L., Bugg, Charles W., Kaltenbach, Linda S., Dunn, Denise, Butland, Stefanie, Weiss, Andreas, Paganetti, Paolo, Lo, Donald C., Patterson, Paul H.
Publicado 2011

Bidirectional Control of Postsynaptic Density-95 (PSD-95) Clustering by Huntingtin

por Parsons, Matthew P., Kang, Rujun, Buren, Caodu, Dau, Alejandro, Southwell, Amber L., Doty, Crystal N., Sanders, Shaun S., Hayden, Michael R., Raymond, Lynn A.
Publicado 2014

Sudden death due to paralysis and synaptic and behavioral deficits when Hip14/Zdhhc17 is deleted in adult mice

por Sanders, Shaun S., Parsons, Matthew P., Mui, Katherine K. N., Southwell, Amber L., Franciosi, Sonia, Cheung, Daphne, Waltl, Sabine, Raymond, Lynn A., Hayden, Michael R.
Publicado en BMC Biol (2016)

Potent and Selective Antisense Oligonucleotides Targeting Single-Nucleotide Polymorphisms in the Huntington Disease Gene / Allele-Specific Silencing of Mutant Huntingtin

por Carroll, Jeffrey B, Warby, Simon C, Southwell, Amber L, Doty, Crystal N, Greenlee, Sarah, Skotte, Niels, Hung, Gene, Bennett, C Frank, Freier, Susan M, Hayden, Michael R
Publicado 2011

Design, Characterization, and Lead Selection of Therapeutic miRNAs Targeting Huntingtin for Development of Gene Therapy for Huntington's Disease

por Miniarikova, Jana, Zanella, Ilaria, Huseinovic, Angelina, van der Zon, Tom, Hanemaaijer, Evelyn, Martier, Raygene, Koornneef, Annemart, Southwell, Amber L, Hayden, Michael R, van Deventer, Sander J, Petry, Harald, Konstantinova, Pavlina
Publicado en Mol Ther Nucleic Acids (2016)

HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo

por Ehrnhoefer, Dagmar E, Southwell, Amber L, Sivasubramanian, Meenalochani, Qiu, Xiaofan, Villanueva, Erika B, Xie, Yuanyun, Waltl, Sabine, Anderson, Lisa, Fazeli, Anita, Casal, Lorenzo, Felczak, Boguslaw, Tsang, Michelle, Hayden, Michael R
Publicado en Hum Mol Genet (2018)

Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease

por Carroll, Jeffrey B, Southwell, Amber L, Graham, Rona K, Lerch, Jason P, Ehrnhoefer, Dagmar E, Cao, Li-Ping, Zhang, Wei-Ning, Deng, Yu, Bissada, Nagat, Henkelman, R Mark, Hayden, Michael R
Publicado 2011

Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry

por Kay, Chris, Collins, Jennifer A, Skotte, Niels H, Southwell, Amber L, Warby, Simon C, Caron, Nicholas S, Doty, Crystal N, Nguyen, Betty, Griguoli, Annamaria, Ross, Colin J, Squitieri, Ferdinando, Hayden, Michael R
Publicado en Mol Ther (2015)

The Interaction of Aging and Cellular Stress Contributes to Pathogenesis in Mouse and Human Huntington Disease Neurons

por Machiela, Emily, Jeloka, Ritika, Caron, Nicholas S., Mehta, Shagun, Schmidt, Mandi E., Baddeley, Helen J. E., Tom, Colton M., Polturi, Nalini, Xie, Yuanyun, Mattis, Virginia B., Hayden, Michael R., Southwell, Amber L.
Publicado en Front Aging Neurosci (2020)

Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS

por Østergaard, Michael E., Southwell, Amber L., Kordasiewicz, Holly, Watt, Andrew T., Skotte, Niels H., Doty, Crystal N., Vaid, Kuljeet, Villanueva, Erika B., Swayze, Eric E., Frank Bennett, C., Hayden, Michael R., Seth, Punit P.
Publicado 2013

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression

por Southwell, Amber L., Smith, Stephen E.P., Davis, Tessa R., Caron, Nicholas S., Villanueva, Erika B., Xie, Yuanyun, Collins, Jennifer A., Li Ye, Min, Sturrock, Aaron, Leavitt, Blair R., Schrum, Adam G., Hayden, Michael R.
Publicado en Sci Rep (2015)

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease

por Ehrnhoefer, Dagmar E., Martin, Dale D. O., Schmidt, Mandi E., Qiu, Xiaofan, Ladha, Safia, Caron, Nicholas S., Skotte, Niels H., Nguyen, Yen T. N., Vaid, Kuljeet, Southwell, Amber L., Engemann, Sabine, Franciosi, Sonia, Hayden, Michael R.
Publicado en Acta Neuropathol Commun (2018)