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Christine Wroge, Joshua Hogins, L. Eisenman, S. Mennerick (2012)
Synaptic NMDA Receptors Mediate Hypoxic Excitotoxic DeathThe Journal of Neuroscience, 32
K. Kołodziejczyk, Matthew Parsons, A. Southwell, M. Hayden, L. Raymond (2014)
Striatal Synaptic Dysfunction and Hippocampal Plasticity Deficits in the Hu97/18 Mouse Model of Huntington DiseasePLoS ONE, 9
K. Huh, R. Wenthold (1999)
Turnover Analysis of Glutamate Receptors Identifies a Rapidly Degraded Pool of the N-Methyl-d-aspartate Receptor Subunit, NR1, in Cultured Cerebellar Granule Cells*The Journal of Biological Chemistry, 274
M. Heine, L. Groc, R. Frischknecht, J. Béïque, B. Lounis, Gavin Rumbaugh, R. Huganir, L. Cognet, D. Choquet (2008)
Surface Mobility of Postsynaptic AMPARs Tunes Synaptic TransmissionScience, 320
Clare Gladding, L. Raymond (2011)
Mechanisms underlying NMDA receptor synaptic/extrasynaptic distribution and functionMolecular and Cellular Neuroscience, 48
M. Difiglia, E. Sapp, K. Chase, S. Davies, G. Bates, J. Vonsattel, N. Aronin (1997)
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.Science, 277 5334
D. Lissin, S. Gomperts, R. Carroll, C. Christine, D. Kalman, Marina Kitamura, S. Hardy, R. Nicoll, R. Malenka, M. Zastrow (1998)
Activity differentially regulates the surface expression of synaptic AMPA and NMDA glutamate receptors.Proceedings of the National Academy of Sciences of the United States of America, 95 12
H. Kordasiewicz, L. Stanek, E. Wancewicz, C. Mazur, Melissa McAlonis, Kimberly A. Pytel, Jonathan Artates, A. Weiss, Seng Cheng, L. Shihabuddin, G. Hung, C. Bennett, D. Cleveland (2012)
Sustained Therapeutic Reversal of Huntington's Disease by Transient Repression of Huntingtin SynthesisNeuron, 74
G. Hardingham, Y. Fukunaga, H. Bading (2002)
Extrasynaptic NMDARs oppose synaptic NMDARs by triggering CREB shut-off and cell death pathwaysNature Neuroscience, 5
C. Fourie, E. Kim, H. Waldvogel, J. Wong, A. McGregor, R. Faull, J. Montgomery (2014)
Differential Changes in Postsynaptic Density Proteins in Postmortem Huntington's Disease and Parkinson's Disease Human BrainsJournal of Neurodegenerative Diseases, 2014
Lijun Li, M. Fan, C. Icton, N. Chen, B. Leavitt, M. Hayden, T. Murphy, L. Raymond (2003)
Role of NR2B-type NMDA receptors in selective neurodegeneration in Huntington diseaseNeurobiology of Aging, 24
Adish Dani, Bo Huang, J. Bergan, C. Dulac, X. Zhuang (2010)
Superresolution Imaging of Chemical Synapses in the BrainNeuron, 68
M. Becher, J. Kotzuk, A. Sharp, S. Davies, G. Bates, D. Price, C. Ross (1998)
Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat LengthNeurobiology of Disease, 4
S. Henley, E. Wild, N. Hobbs, R. Scahill, G. Ridgway, D. MacManus, R. Barker, Nick Fox, S. Tabrizi (2009)
Relationship between CAG repeat length and brain volume in premanifest and early Huntington’s diseaseJournal of Neurology, 256
C. Gutekunst, Shihua Li, Hong Yi, James Mulroy, S. Kuemmerle, Randi Jones, David Rye, R. Ferrante, S. Hersch, Xiao-Jiang Li (1999)
Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to NeuropathologyThe Journal of Neuroscience, 19
Xiaobing Chen, C. Winters, R. Azzam, Xiang Li, J. Galbraith, R. Leapman, T. Reese (2008)
Organization of the core structure of the postsynaptic densityProceedings of the National Academy of Sciences, 105
D. Baddeley, D. Crossman, S. Rossberger, J. Cheyne, J. Montgomery, Isuru Jayasinghe, C. Cremer, M. Cannell, C. Soeller (2011)
4D Super-Resolution Microscopy with Conventional Fluorophores and Single Wavelength Excitation in Optically Thick Cells and TissuesPLoS ONE, 6
H. Monyer, N. Burnashev, D. Laurie, B. Sakmann, P. Seeburg (1994)
Developmental and regional expression in the rat brain and functional properties of four NMDA receptorsNeuron, 12
A. M. Craig (1998)
Culturing nerve cells
Charity Aiken, A. Tobin, E. Schweitzer (2004)
A cell-based screen for drugs to treat Huntington's diseaseNeurobiology of Disease, 16
G. Hardingham, H. Bading (2010)
Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disordersNature Reviews Neuroscience, 11
C. Waites, C. Specht, Kai Härtel, Sergio Leal-Ortiz, D. Genoux, Dong Li, R. Drisdel, Okunola Jeyifous, J. Cheyne, W. Green, J. Montgomery, C. Garner (2009)
Synaptic SAP97 Isoforms Regulate AMPA Receptor Dynamics and Access to Presynaptic GlutamateThe Journal of Neuroscience, 29
M. Levine, G. Klapstein, Ahrin Koppel, E. Gruen, C. Cepeda, M. Vargas, E. Jokel, E. Carpenter, H. Zanjani, R. Hurst, A. Efstratiadis, S. Zeitlin, M. Chesselet (1999)
Enhanced sensitivity to N‐methyl‐D‐aspartate receptor activation in transgenic and knockin mouse models of Huntington's diseaseJournal of Neuroscience Research, 58
R. O’Brien, Sunjeev Kamboj, M. Ehlers, Kenneth Rosen, G. Fischbach, R. Huganir (1998)
Activity-Dependent Modulation of Synaptic AMPA Receptor AccumulationNeuron, 21
R. Ventura, K. Harris (1999)
Three-Dimensional Relationships between Hippocampal Synapses and AstrocytesThe Journal of Neuroscience, 19
I. Al-Ramahi, S. Giridharan, Yu-Chi Chen, S. Patnaik, N. Safren, Junya Hasegawa, María Haro, Amanda Gee, S. Titus, Hyunkyung Jeong, J. Clarke, D. Krainc, Wei Zheng, R. Irvine, S. Barmada, M. Ferrer, Noel Southall, L. Weisman, J. Botas, J. Marugan (2017)
Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin proteineLife, 6
M. Köhrmann, W. Haubensak, Indradeo Hemraj, C. Kaether, V. Lessmann, M. Kiebler (1999)
Fast, convenient, and effective method to transiently transfect primary hippocampal neuronsJournal of Neuroscience Research, 58
Bryan Jarabek, R. Yasuda, B. Wolfe (2003)
Regulation of proteins affecting NMDA receptor-induced excitotoxicity in a Huntington's mouse model.Brain : a journal of neurology, 127 Pt 3
Thomas Papouin, Laurent Ladépêche, Jérôme Ruel, S. Sacchi, Marilyne Labasque, Marwa Hanini, L. Groc, L. Pollegioni, J. Mothet, S. Oliet (2012)
Synaptic and Extrasynaptic NMDA Receptors Are Gated by Different Endogenous CoagonistsCell, 150
I. Bohanna, N. Georgiou‐Karistianis, A. Hannan, G. Egan (2008)
Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington's diseaseBrain Research Reviews, 58
V. André, C. Cepeda, A. Venegas, Y. Gomez, M. Levine (2006)
Altered cortical glutamate receptor function in the R6/2 model of Huntington's disease.Journal of neurophysiology, 95 4
A. Molero, Eduardo Arteaga-Bracho, Christopher Chen, M. Gulinello, Michael Winchester, N. Pichamoorthy, Solen Gokhan, K. Khodakhah, M. Mehler (2016)
Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington’s diseaseProceedings of the National Academy of Sciences, 113
Emma Scotter, Catherine Goodfellow, E. Graham, M. Dragunow, M. Glass (2010)
Neuroprotective potential of CB1 receptor agonists in an in vitro model of Huntington's diseaseBritish Journal of Pharmacology, 160
Jing Fan, C. Cowan, Lily Zhang, M. Hayden, L. Raymond (2009)
Interaction of Postsynaptic Density Protein-95 with NMDA Receptors Influences Excitotoxicity in the Yeast Artificial Chromosome Mouse Model of Huntington's DiseaseThe Journal of Neuroscience, 29
O. Schlüter, Weifeng Xu, R. Malenka (2006)
Alternative N-Terminal Domains of PSD-95 and SAP97 Govern Activity-Dependent Regulation of Synaptic AMPA Receptor FunctionNeuron, 51
Okunola Jeyifous, C. Waites, C. Specht, S. Fujisawa, M. Schubert, Eric Lin, J. Marshall, C. Aoki, Tharani Silva, J. Montgomery, C. Garner, W. Green (2009)
SAP97 and CASK mediate sorting of N-Methyl-D-Aspartate Receptors through a novel secretory pathwayNature neuroscience, 12
Karim Meur, Micaela Galante, M. Angulo, E. Audinat (2007)
Tonic activation of NMDA receptors by ambient glutamate of non‐synaptic origin in the rat hippocampusThe Journal of Physiology, 580
Kevin Lee, Lucy Goodman, C. Fourie, S. Schenk, B. Leitch, J. Montgomery (2016)
AMPA Receptors as Therapeutic Targets for Neurological Disorders.Advances in protein chemistry and structural biology, 103
S. Suhr, E. Gil, M. Senut, F. Gage (1998)
High level transactivation by a modified Bombyx ecdysone receptor in mammalian cells without exogenous retinoid X receptor.Proceedings of the National Academy of Sciences of the United States of America, 95 14
(1994)
Differential activation of CREB by Ca/calmodulin-dependent protein kinases type II
M. Fan, L. Raymond (2007)
N-Methyl-d-aspartate (NMDA) receptor function and excitotoxicity in Huntington's diseaseProgress in Neurobiology, 81
M. Tokunaga, N. Imamoto, K. Sakata-Sogawa (2008)
Highly inclined thin illumination enables clear single-molecule imaging in cellsNature Methods, 5
A. Reiner, I. Dragatsis, P. Dietrich (2011)
Genetics and neuropathology of Huntington's disease.International review of neurobiology, 98
(1998)
Transfecting cultured neurons
Y. Sun, Anneli Savanenin, P. Reddy, Y. Liu (2001)
Polyglutamine-expanded Huntingtin Promotes Sensitization of N-Methyl-d-aspartate Receptors via Post-synaptic Density 95*The Journal of Biological Chemistry, 276
(1993)
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's disease collaborative research group, 72
G. Collingridge, J. Watkins (1995)
The NMDA Receptor
S. Kaech, G. Banker (2006)
Culturing hippocampal neuronsNature Protocols, 1
Magali Arons, Kevin Lee, Charlotte Thynne, SallyA Kim, Claudia Schob, S. Kindler, J. Montgomery, C. Garner (2016)
Shank3 Is Part of a Zinc-Sensitive Signaling System That Regulates Excitatory Synaptic StrengthThe Journal of Neuroscience, 36
A. Milnerwood, A. Kaufman, Marja Sepers, Clare Gladding, Lily Zhang, Liang Wang, Jing Fan, Ainsley Coquinco, Joy Qiao, Hwan-Bong Lee, Y. Wang, M. Cynader, L. Raymond (2012)
Mitigation of augmented extrasynaptic NMDAR signaling and apoptosis in cortico-striatal co-cultures from Huntington's disease miceNeurobiology of Disease, 48
S. Okamoto, M. Pouladi, M. Talantova, Dongdong Yao, Peng Xia, Dagmar Ehrnhoefer, Rameez Zaidi, Arjay Clemente, M. Kaul, R. Graham, Dongxian Zhang, H. Chen, Gary Tong, M. Hayden, S. Lipton (2009)
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtinNature medicine, 15
M. Usdin, M. Usdin, P. Shelbourne, R. Myers, D. Madison, D. Madison (1999)
Impaired synaptic plasticity in mice carrying the Huntington's disease mutation.Human molecular genetics, 8 5
Dong Li, C. Specht, C. Waites, Charlotte Butler-Munro, Sergio Leal-Ortiz, Janie Foote, D. Genoux, C. Garner, J. Montgomery (2011)
SAP97 directs NMDA receptor spine targeting and synaptic plasticityThe Journal of Physiology, 589
G. Yudowski, M. Puthenveedu, D. Leonoudakis, S. Panicker, K. Thorn, E. Beattie, M. Zastrow (2007)
Real-Time Imaging of Discrete Exocytic Events Mediating Surface Delivery of AMPA ReceptorsThe Journal of Neuroscience, 27
K. Matsui, C. Jahr, M. Rubio (2005)
High-Concentration Rapid Transients of Glutamate Mediate Neural-Glial Communication via Ectopic ReleaseThe Journal of Neuroscience, 25
H. MacGillavry, Yu Song, S. Raghavachari, T. Blanpied (2013)
Nanoscale Scaffolding Domains within the Postsynaptic Density Concentrate Synaptic AMPA ReceptorsNeuron, 78
M. Mandal, Jing Wei, P. Zhong, Jia Cheng, Lara Duffney, Wenhua Liu, E. Yuen, Alison Twelvetrees, Shihua Li, Xiao-Jiang Li, J. Kittler, Zhen Yan (2011)
Impaired α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) Receptor Trafficking and Function by Mutant Huntingtin*The Journal of Biological Chemistry, 286
C. Cepeda, M. Ariano, Christopher Calvert, J. Flores-Hernández, S. Chandler, B. Leavitt, M. Hayden, M. Levine (2001)
NMDA receptor function in mouse models of Huntington diseaseJournal of Neuroscience Research, 66
J. Cheyne, J. Montgomery (2008)
Plasticity-dependent changes in metabotropic glutamate receptor expression at excitatory hippocampal synapsesMolecular and Cellular Neuroscience, 37
Jian Shi, Matthew Townsend, M. Constantine‐Paton (2000)
Activity-Dependent Induction of Tonic Calcineurin Activity Mediates a Rapid Developmental Downregulation of NMDA Receptor CurrentsNeuron, 28
Jing Fan, Clare Gladding, Liang Wang, Lily Zhang, A. Kaufman, A. Milnerwood, L. Raymond (2012)
P38 MAPK is involved in enhanced NMDA receptor-dependent excitotoxicity in YAC transgenic mouse model of Huntington diseaseNeurobiology of Disease, 45
S. Vicini, Jian Wang, Jinhong Li, W. Zhu, Yue‐hua Wang, Jian‐hong Luo, B. Wolfe, D. Grayson (1998)
Functional and pharmacological differences between recombinant N-methyl-D-aspartate receptors.Journal of neurophysiology, 79 2
Peiqing Sun, H. Enslen, P. Myung, R. Maurer (1994)
Differential activation of CREB by Ca2+/calmodulin-dependent protein kinases type II and type IV involves phosphorylation of a site that negatively regulates activity.Genes & development, 8 21
A. Milnerwood, D. Cummings, G. Dallérac, Jacki Brown, Sarat Vatsavayai, M. Hirst, P. Rezaie, K. Murphy (2006)
Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease.Human molecular genetics, 15 10
M. Howard, G. Elias, Laura Elias, W. Swat, R. Nicoll (2010)
The role of SAP97 in synaptic glutamate receptor dynamicsProceedings of the National Academy of Sciences, 107
Jun Wu, Daniel Ryskamp, X. Liang, P. Egorova, O. Zakharova, G. Hung, I. Bezprozvanny (2016)
Enhanced Store-Operated Calcium Entry Leads to Striatal Synaptic Loss in a Huntington's Disease Mouse ModelThe Journal of Neuroscience, 36
Magali Arons, Charlotte Thynne, A. Grabrucker, Dong Li, Michael Schoen, J. Cheyne, T. Boeckers, J. Montgomery, C. Garner (2012)
Autism-Associated Mutations in ProSAP2/Shank3 Impair Synaptic Transmission and Neurexin–Neuroligin-Mediated Transsynaptic SignalingThe Journal of Neuroscience, 32
N. Chen, Tao Luo, C. Wellington, M. Metzler, K. McCutcheon, M. Hayden, L. Raymond (1999)
Subtype‐Specific Enhancement of NMDA Receptor Currents by Mutant HuntingtinJournal of Neurochemistry, 72
O. Jeyifous, C. L. Waites, C. G. Specht, S. Fujisawa, M. Schubert, E. I. Lin, W. N. Green (2009)
SAP97 and CASK mediate sorting of NMDA receptors through a previously unknown secretory pathway, 12
Qiang Zhou, Morgan Sheng (2013)
NMDA receptors in nervous system diseasesNeuropharmacology, 74
K. Murphy, R. Carter, Lisa Lione, L. Mangiarini, A. Mahal, G. Bates, Stephen Dunnett, A. Morton (2000)
Abnormal Synaptic Plasticity and Impaired Spatial Cognition in Mice Transgenic for Exon 1 of the Human Huntington's Disease MutationThe Journal of Neuroscience, 20
Melinda Zeron, O. Hansson, N. Chen, C. Wellington, B. Leavitt, P. Brundin, M. Hayden, L. Raymond (2002)
Increased Sensitivity to N-Methyl-D-Aspartate Receptor-Mediated Excitotoxicity in a Mouse Model of Huntington's DiseaseNeuron, 33
R. Petralia, N. Sans, Ya‐Xian Wang, R. Wenthold (2005)
Ontogeny of postsynaptic density proteins at glutamatergic synapsesMolecular and Cellular Neuroscience, 29
R. Petralia (2012)
Distribution of Extrasynaptic NMDA Receptors on NeuronsThe Scientific World Journal, 2012
D. Simmons, R. Mehta, J. Lauterborn, C. Gall, G. Lynch (2011)
Brief ampakine treatments slow the progression of Huntington's disease phenotypes in R6/2 miceNeurobiology of Disease, 41
N. Sans, R. Petralia, Ya‐Xian Wang, J. Blahoš, J. Hell, R. Wenthold (2000)
A Developmental Change in NMDA Receptor-Associated Proteins at Hippocampal SynapsesThe Journal of Neuroscience, 20
S. Cull-Candy, Daniel Leszkiewicz (2004)
Role of Distinct NMDA Receptor Subtypes at Central SynapsesScience's STKE, 2004
Laurent Groc, Lucie Bard, Daniel Choquet (2009)
Surface trafficking of N-methyl-d-aspartate receptors: Physiological and pathological perspectivesNeuroscience, 158
J. Carroll, S. Warby, A. Southwell, Crystal Doty, Sarah Greenlee, N. Skotte, N. Skotte, G. Hung, C. Bennett, S. Freier, M. Hayden (2011)
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 12
A. Karpova, M. Mikhaylova, S. Bera, Julia Bär, P. Reddy, T. Behnisch, V. Rankovic, C. Spilker, P. Bethge, Jale Sahin, R. Kaushik, W. Zuschratter, T. Kähne, M. Naumann, E. Gundelfinger, M. Kreutz (2013)
Encoding and Transducing the Synaptic or Extrasynaptic Origin of NMDA Receptor Signals to the NucleusCell, 152
Lucy Goodman, D. Baddeley, Wojciech Ambroziak, C. Waites, C. Garner, C. Soeller, J. Montgomery (2017)
N‐terminal SAP97 isoforms differentially regulate synaptic structure and postsynaptic surface pools of AMPA receptorsHippocampus, 27
D. Baddeley, M. Cannell, C. Soeller (2010)
Visualization of Localization Microscopy DataMicroscopy and Microanalysis, 16
J. Cha, C. Kosinski, J. Kerner, S. Alsdorf, L. Mangiarini, S. Davies, J. Penney, G. Bates, A. Young (1998)
Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene.Proceedings of the National Academy of Sciences of the United States of America, 95 11
M. Wagster, J. Hedreen, C. Peyser, S. Folstein, C. Ross (1994)
Selective Loss of [3H]Kainic Acid and [3H]AMPA Binding in Layer VI of Frontal Cortex in Huntington's DiseaseExperimental Neurology, 127
K. Tovar, G. Westbrook (2002)
Mobile NMDA Receptors at Hippocampal SynapsesNeuron, 34
S. Davies, M. Turmaine, Barbara Cozens, M. Difiglia, A. Sharp, C. Ross, E. Scherzinger, E. Wanker, L. Mangiarini, G. Bates (1997)
Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD MutationCell, 90
M. Kukley, E. Capetillo-Zarate, D. Dietrich (2007)
Vesicular glutamate release from axons in white matterNature Neuroscience, 10
D. Simmons, Christopher Rex, L. Palmer, V. Pandyarajan, V. Fedulov, C. Gall, G. Lynch (2009)
Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin miceProceedings of the National Academy of Sciences, 106
C. Tardin, L. Cognet, C. Bats, B. Lounis, D. Choquet (2003)
Direct imaging of lateral movements of AMPA receptors inside synapsesThe EMBO Journal, 22
Hiroshi Makino, R. Malinow (2009)
AMPA Receptor Incorporation into Synapses during LTP: The Role of Lateral Movement and ExocytosisNeuron, 64
J. Montgomery, P. Zamorano, C. Garner (2004)
MAGUKs in synapse assembly and function: an emerging viewCellular and Molecular Life Sciences CMLS, 61
Olivia Shipton, O. Paulsen (2014)
GluN2A and GluN2B subunit-containing NMDA receptors in hippocampal plasticityPhilosophical Transactions of the Royal Society B: Biological Sciences, 369
J. Torres-Peraza, A. Giralt, J. García-Martínez, E. Pedrosa, J. Canals, J. Alberch (2008)
Disruption of striatal glutamatergic transmission induced by mutant huntingtin involves remodeling of both postsynaptic density and NMDA receptor signalingNeurobiology of Disease, 29
H. Man, Jerry Lin, W. Ju, G. Ahmadian, Lidong Liu, L. Becker, M. Sheng, Y. Wang (2000)
Regulation of AMPA Receptor–Mediated Synaptic Transmission by Clathrin-Dependent Receptor InternalizationNeuron, 25
G. Laforet, E. Sapp, K. Chase, Charmian McIntyre, F. Boyce, M. Campbell, Beth Cadigan, Lori Warzecki, D. Tagle, P. Reddy, C. Cepeda, Christopher Calvert, E. Jokel, G. Klapstein, M. Ariano, M. Levine, M. Difiglia, N. Aronin, Steven Jones, Janet Stein (2001)
Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's DiseaseThe Journal of Neuroscience, 21
Matthew Townsend, Yudong Liu, M. Constantine‐Paton (2004)
Retina-Driven Dephosphorylation of the NR2A Subunit Correlates with Faster NMDA Receptor Kinetics at Developing Retinocollicular SynapsesThe Journal of Neuroscience, 24
S. Cull-Candy, S. Brickley, M. Farrant (2001)
NMDA receptor subunits: diversity, development and diseaseCurrent Opinion in Neurobiology, 11
M. MacDonald, C. Ambrose, M. Duyao, R. Myers, Carol Lin, L. Srinidhi, G. Barnes, Sherryl Taylor, M. James, Nicolet Groot, Heather Macfarlane, B. Jenkins, M. Anderson, N. Wexler, J. Gusella, G. Bates, S. Baxendale, H. Hummerich, S. Kirby, M. North, S. Youngman, R. Mott, G. Zehetner, Z. Sedlacek, A. Poustka, A. Frischauf, H. Lehrach, A. Buckler, D. Church, L. Doucette-Stamm, M. O’Donovan, Laura Riba-Ramírez, Manish Shah, V. Stanton, S. Strobel, K. Draths, Jennifer Wales, P. Dervan, D. Housman, M. Altherr, R. Shiang, L. Thompson, T. Fielder, J. Wasmuth, D. Tagle, J. Valdes, Lon Elmer, M. Allard, L. Castilla, M. Swaroop, K. Blanchard, F. Collins, R. Snell, T. Holloway, Kathleen Gillespie, N. Datson, D. Shaw, P. Harper (1993)
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomesCell, 72
J. Kang, J. Frank, Chang Kang, H. Kajiura, Meenu Vikram, Akihiro Ueda, Sewon Kim, J. Bahk, Barbara Triplett, K. Fujiyama, Sang Lee, A. Schaewen, H. Koiwa (2008)
Organization of the core structure of the postsynaptic density
J. Hodgson, N. Agopyan, C. Gutekunst, B. Leavitt, F. LePiane, R. Singaraja, Desmond Smith, N. Bissada, K. McCutcheon, J. Nasir, L. Jamot, Xiao-Jiang Li, M. Stevens, Erica Rosemond, J. Roder, A. Phillips, E. Rubin, S. Hersch, M. Hayden (1999)
A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal NeurodegenerationNeuron, 23
A. Milnerwood, Clare Gladding, M. Pouladi, A. Kaufman, R. Hines, J. Boyd, R. Ko, O. Vasuta, R. Graham, M. Hayden, T. Murphy, L. Raymond (2010)
Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease MiceNeuron, 65
Huntington's disease (HD) is a genetic neurodegenerative disorder caused by an expansion of the CAG repeat tract in the HTT gene, leading to motor, cognitive, and psychiatric symptoms. At the cellular level, NMDA‐type glutamate receptors are upregulated at glutamatergic extrasynaptic sites in HD, triggering cell death signaling pathways and driving HD neurodegeneration. Extrasynaptic and synaptic glutamate receptor trafficking and surface distribution are regulated by the α and β N‐terminal isoforms of SAP97, a postsynaptic density protein localized at glutamatergic synapses. Here we examined the surface distribution of NMDARs and AMPARs in a cellular model of HD, and whether the manipulation of individual SAP97 isoforms can regulate receptor distribution in diseased neurons. Using dSTORM super‐resolution imaging, we reveal that mutant HTT drives the elevation of extrasynaptic NMDAR clusters located 100–500 nm from the postsynaptic density. This was accompanied by a decline in synaptic NMDAR‐mediated currents while surface NMDAR‐mediated currents remained unchanged. These effects were induced within 3 days of mutant HTT expression in rat hippocampal neurons in vitro, and were specific for NMDARs and not observed with AMPARs. Intriguingly, upregulation of either α‐ or βSAP97 expression increased synaptic and/or perisynaptic NMDAR localization and prevented the shift of NMDARs to extrasynaptic sites in mutant HTT neurons. This was accompanied by the rescue of normal synaptic NMDAR‐mediated currents. Taken together, our high‐resolution data reveals plasticity in surface NMDAR localization driven by mutant HTT and identifies the similar but independent roles of SAP97 N‐terminal isoforms in maintaining normal synaptic function in pathological states.
Hippocampus – Wiley
Published: Oct 1, 2018
Keywords: ; ; ; ;
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