Biological Aging and the Cellular Pathogenesis of Huntington’s Disease

door Machiela, Emily, Southwell, Amber L.
Gepubliceerd in J Huntingtons Dis (2020)

Gene Therapy in Mouse Models of Huntington Disease

door Southwell, Amber L., Patterson, Paul H.
Gepubliceerd in 2011

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

door Southwell, Amber L., Ko, Jan, Patterson, Paul H.
Gepubliceerd in 2009

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

door Bedwell, Jeffrey S., Spencer, Christopher C., Southwell, Amber L.
Gepubliceerd in Psychopathology (2020)

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

door Zylka, Mark J., Dong, Xinzhong, Southwell, Amber L., Anderson, David J.
Gepubliceerd in 2003

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

door Kolodziejczyk, Karolina, Parsons, Matthew P., Southwell, Amber L., Hayden, Michael R., Raymond, Lynn A.
Gepubliceerd in 2014

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

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

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

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

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

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

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

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

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

door 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.
Gepubliceerd in BMC Biol (2016)

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

door 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
Gepubliceerd in 2011

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

door 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
Gepubliceerd in Mol Ther Nucleic Acids (2016)

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

door 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
Gepubliceerd in Hum Mol Genet (2018)

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

door 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
Gepubliceerd in 2011

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

door 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
Gepubliceerd in Mol Ther (2015)

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

door 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.
Gepubliceerd in 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

door 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.
Gepubliceerd in 2013

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

door 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.
Gepubliceerd in Sci Rep (2015)

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

door 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.
Gepubliceerd in Acta Neuropathol Commun (2018)