Search Results - "Graham, Rona K"

Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice by Milnerwood, Austen J., Gladding, Clare M., Pouladi, Mahmoud A., Kaufman, Alexandra M., Hines, Rochelle M., Boyd, Jamie D., Ko, Rebecca W.Y., Vasuta, Oana C., Graham, Rona K., Hayden, Michael R., Murphy, Timothy H., Raymond, Lynn A.

“…N-methyl-D-aspartate receptor (NMDAR) excitotoxicity is implicated in the pathogenesis of Huntington's disease (HD), a late-onset neurodegenerative disorder…”
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Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin by Talantova, Maria, Kaul, Marcus, Yao, Dongdong, Xia, Peng, Ehrnhoefer, Dagmar E, Zaidi, Rameez, Lipton, Stuart A, Graham, Rona K, Tong, Gary, Zhang, Dongxian, Clemente, Arjay, Okamoto, Shu-ichi, Hayden, Michael R, Pouladi, Mahmoud A, Vincent Chen, H-S

“…In a mouse model of Huntington's disease, synaptic activation of NMDA receptors induces the formation of huntingtin-containing inclusions, rendering neurons…”
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Cleavage at the Caspase-6 Site Is Required for Neuronal Dysfunction and Degeneration Due to Mutant Huntingtin by Graham, Rona K., Deng, Yu, Slow, Elizabeth J., Haigh, Brendan, Bissada, Nagat, Lu, Ge, Pearson, Jacqueline, Shehadeh, Jacqueline, Bertram, Lisa, Murphy, Zoe, Warby, Simon C., Doty, Crystal N., Roy, Sophie, Wellington, Cheryl L., Leavitt, Blair R., Raymond, Lynn A., Nicholson, Donald W., Hayden, Michael R.

“…Cleavage of huntingtin (htt) has been characterized in vitro, and accumulation of caspase cleavage fragments represents an early pathological change in brains…”
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Activation of caspase‐6 and cleavage of caspase‐6 substrates is an early event in NMDA receptor–mediated excitotoxicity by Girling, Kimberly D., Demers, Marie‐Josee, Laine, Jean, Zhang, Shu, Wang, Yu Tian, Graham, Rona K.

“…Excitotoxicity, due to overstimulation of N‐methyl D‐aspartate receptors (NMDARs), has a pivotal role in many neurological disorders. However, NMDAR…”
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Characterization of age-associated changes in peripheral organ and brain region weights in C57BL/6 mice by Lessard-Beaudoin, Mélissa, Laroche, Mélissa, Demers, Marie-Josée, Grenier, Guillaume, Graham, Rona K.

“…In order to further understand age-related physiological changes and to have in depth reference values for C57BL/6 mice, we undertook a study to assess the…”
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Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice by Wong, Bibiana K.Y, Ehrnhoefer, Dagmar E, Graham, Rona K, Martin, Dale D.O, Ladha, Safia, Uribe, Valeria, Stanek, Lisa M, Franciosi, Sonia, Qiu, Xiaofan, Deng, Yu, Kovalik, Vlad, Zhang, Weining, Pouladi, Mahmoud A, Shihabuddin, Lamya S, Hayden, Michael R

“…Abstract Huntington Disease (HD) is a progressive neurodegenerative disease caused by an elongated CAG repeat in the huntingtin ( HTT ) gene that encodes a…”
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Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease by Zhang, Hua, Li, Qin, Graham, Rona K, Slow, Elizabeth, Hayden, Michael R, Bezprozvanny, Ilya

“…Abstract Huntington's disease (HD) is caused by a progressive loss of striatal medium spiny neurons (MSN). The molecular trigger of HD is a polyglutamine…”
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Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease by Wang, Chuan-En, Tydlacka, Suzanne, Orr, Adam L., Yang, Shang-Hsun, Graham, Rona K., Hayden, Michael R., Li, Shihua, Chan, Anthony W.S., Li, Xiao-Jiang

“…A number of mouse models expressing mutant huntingtin (htt) with an expanded polyglutamine (polyQ) domain are useful for studying the pathogenesis of…”
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Absence of Behavioral Abnormalities and Neurodegeneration in vivo despite Widespread Neuronal Huntingtin Inclusions by Slow, Elizabeth J., Graham, Rona K., Osmand, Alexander P., Devon, Rebecca S., Lu, Ge, Deng, Yu, Pearson, Jacqui, Vaid, Kuljeet, Bissada, Nagat, Wetzel, Ronald, Leavitt, Blair R., Hayden, Michael R., Palmiter, Richard D.

“…We have serendipitously established a mouse that expresses an N-terminal human huntingtin (htt) fragment with an expanded polyglutamine repeat (≈120) under the…”
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Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice by URIBE, Valeria, WONG, Bibiana K. Y, YU DENG, FRANCIOSI, Sonia, BISSADA, Nagat, SPREEUW, Amanda, WEINING ZHANG, EHRNHOEFER, Dagmar E, VAID, Kuljeet, MILLER, Freda D, DESHMUKH, Mohanish, HOWLAND, David, GRAHAM, Rona K, HARDEN, Michael R, CUSACK, Corey L, SKOTTE, Niels H, POULADI, Mahmoud A, YUANYUN XIE, FEINBERG, Konstantin, YIMIAO OU, YINGBIN OUYANG

“…Apoptosis, or programmed cell death, is a cellular pathway involved in normal cell turnover, developmental tissue remodeling, embryonic development, cellular…”
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Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin by Waldron-Roby, Elaine, Ratovitski, Tamara, Wang, XiaoFang, Jiang, Mali, Watkin, Erin, Arbez, Nikolas, Graham, Rona K, Hayden, Michael R, Hou, Zhipeng, Mori, Susumu, Swing, Deborah, Pletnikov, Mikhail, Duan, Wenzhen, Tessarollo, Lino, Ross, Christopher A

“…Huntington's disease (HD) is caused by a polyglutamine expansion in the Huntingtin (Htt) protein. Proteolytic cleavage of Htt into toxic N-terminal fragments…”
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NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease by Pouladi, Mahmoud A, Brillaud, Elsa, Xie, Yuanyun, Conforti, Paola, Graham, Rona K, Ehrnhoefer, Dagmar E, Franciosi, Sonia, Zhang, Weining, Poucheret, Patrick, Compte, Elsa, Maurel, Jean-Claude, Zuccato, Chiara, Cattaneo, Elena, Néri, Christian, Hayden, Michael R

“…Abstract Huntington disease (HD), a neurodegenerative disorder caused by an expanded CAG repeat in the HTT gene, remains without a treatment to modify the…”
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Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments by Warby, Simon C., Doty, Crystal N., Graham, Rona K., Shively, Jonathan, Singaraja, Roshni R., Hayden, Michael R.

“…Huntingtin is phosphorylated on serine-421 (S421) by the pro-survival signaling protein kinases Akt and SGK. Phosphorylation of huntingtin at S421 is variable…”
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Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity by Stroedicke, Martin, Bounab, Yacine, Strempel, Nadine, Klockmeier, Konrad, Yigit, Sargon, Friedrich, Ralf P, Chaurasia, Gautam, Li, Shuang, Hesse, Franziska, Riechers, Sean-Patrick, Russ, Jenny, Nicoletti, Cecilia, Boeddrich, Annett, Wiglenda, Thomas, Haenig, Christian, Schnoegl, Sigrid, Fournier, David, Graham, Rona K, Hayden, Michael R, Sigrist, Stephan, Bates, Gillian P, Priller, Josef, Andrade-Navarro, Miguel A, Futschik, Matthias E, Wanker, Erich E

“…Assemblies of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological hallmark of Huntington's disease (HD). The molecular…”
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Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice by Guidetti, Paolo, Bates, Gillian P., Graham, Rona K., Hayden, Michael R., Leavitt, Blair R., MacDonald, Marcy E., Slow, Elizabeth J., Wheeler, Vanessa C., Woodman, Ben, Schwarcz, Robert

“…The brain levels of the endogenous excitotoxin quinolinic acid (QUIN) and its bioprecursor, the free radical generator 3-hydroxykynurenine (3-HK), are elevated…”
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Wild‐type huntingtin protects neurons from excitotoxicity by Leavitt, Blair R., Raamsdonk, Jeremy M., Shehadeh, Jacqueline, Fernandes, Herman, Murphy, Zoe, Graham, Rona K., Wellington, Cheryl L., Hayden, Michael R.

“…Huntingtin is a caspase substrate, and loss of normal huntingtin function resulting from caspase‐mediated proteolysis may play a role in the pathogenesis of…”
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Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain by Van Raamsdonk, Jeremy M, Metzler, Martina, Slow, Elizabeth, Pearson, Jacqueline, Schwab, Claudia, Carroll, Jeffrey, Graham, Rona K, Leavitt, Blair R, Hayden, Michael R

“…Abstract The YAC128 mouse model of Huntington disease (HD) exhibits motor abnormalities, cognitive dysfunction and selective neuropathology which are similar…”
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Polyglutamine-Modulated Striatal Calpain Activity in YAC Transgenic Huntington Disease Mouse Model: Impact on NMDA Receptor Function and Toxicity by Cowan, Catherine M, Fan, Mannie M. Y, Fan, Jing, Shehadeh, Jacqueline, Zhang, Lily Y. J, Graham, Rona K, Hayden, Michael R, Raymond, Lynn A

“…Huntington disease (HD), caused by CAG expansion in the ubiquitously expressed huntingtin gene, is characterized by early dysfunction and death of striatal…”
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Caspase Cleavage of Mutant Huntingtin Precedes Neurodegeneration in Huntington's Disease by Wellington, Cheryl L, Ellerby, Lisa M, Gutekunst, Claire-Anne, Rogers, Danny, Warby, Simon, Graham, Rona K, Loubser, Odell, van Raamsdonk, Jeremy, Singaraja, Roshni, Yang, Yu-Zhou, Gafni, Juliette, Bredesen, Dale, Hersch, Steven M, Leavitt, Blair R, Roy, Sophie, Nicholson, Donald W, Hayden, Michael R

“…Huntington's disease (HD) results from polyglutamine expansion in huntingtin (htt), a protein with several consensus caspase cleavage sites. Despite the…”
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Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease by Shehadeh, Jacqueline, Fernandes, Herman B., Zeron Mullins, Melinda M., Graham, Rona K., Leavitt, Blair R., Hayden, Michael R., Raymond, Lynn A.

“…Huntington disease (HD), caused by expansion >35 of a polyglutamine tract in huntingtin, results in degeneration of striatal medium spiny neurons (MSNs)…”
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