Biological Aging and the Cellular Pathogenesis of Huntington’s Disease

av Machiela, Emily, Southwell, Amber L.
I publikationen J Huntingtons Dis (2020)

Gene Therapy in Mouse Models of Huntington Disease

av Southwell, Amber L., Patterson, Paul H.
Publicerad 2011

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

av Southwell, Amber L., Ko, Jan, Patterson, Paul H.
Publicerad 2009

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

av Bedwell, Jeffrey S., Spencer, Christopher C., Southwell, Amber L.
I publikationen Psychopathology (2020)

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

av Zylka, Mark J., Dong, Xinzhong, Southwell, Amber L., Anderson, David J.
Publicerad 2003

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

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

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

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

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

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

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

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

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

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

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

av 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.
I publikationen BMC Biol (2016)

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

av 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
Publicerad 2011

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

av 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
I publikationen Mol Ther Nucleic Acids (2016)

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

av 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
I publikationen Hum Mol Genet (2018)

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

av 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
Publicerad 2011

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

av 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
I publikationen Mol Ther (2015)

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

av 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.
I publikationen 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

av 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.
Publicerad 2013

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

av 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.
I publikationen Sci Rep (2015)

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

av 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.
I publikationen Acta Neuropathol Commun (2018)