Cold Spring Harbor Symposia on Quantitative Biology
LXI: Function & Dysfunction in the Nervous System
1996
CSHL Digital Photo Archives
1996




From left: Dr. Eric Kandel, Dr. Miranda Robertson, Dr. Bruce Stillman



SYMPOSIUM SYNOPSIS

LIST OF PARTICIPANTS

CONTENTS OF SYMPOSIUM VOLUME

PHOTOGRAPHS

PUBLISHED VOLUME








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Function & Dysfunction in the Nervous System

Symposium Volume LXI Contents

Symposium Participants    v

Foreword    xvii



Language and Visual Processing

Cortical Plasticity Underlying Perceptual, Motor, and Cognitive Skill Development: Implications for Neurorehabilitation M.M. Merzenich, B. Wright, W. Jenkins, C. Xerri, N. Byl, S. Miller, and P. Tallal    1

What Words Are Telling Us about the Brain M.E. Raichle    9

Neural Mechanisms for Heading and Structure-from-motion Perception R.A. Andersen, D.C. Bradley, and K.V. Shenoy    15

Visual Responses in Vl of Freely Viewing Monkeys M.S. Livingstone, D.C. Freeman, and D.H. Hubel    27

Amblyopia: A Developmental Disorder of the Central Visual Pathways L. Kiorpes and J.A. Movshon    39

The Functional Anatomy of Visual Awareness C. Koch and J. Braun    49

Handedness A Single Locus, RGHT, Specifies Preference for Hand Utilization in Humans A.J.S. Klar    59



Perceptual Plasticity

Functions and Roles of Glutamate Receptors in Synaptic Transmission and Plasticity S. Nakanishi, Y. Nakajima, A. Nomura, M. Masu, H. Iwakabe, Y. Hayashi, and M. Yokoi    67

Combination of Gene Targeting and Gene Transfer by Adenoviral Vectors in the Analysis of Neurotrophin-mediated Neuronal Plasticity O. Griesbeck, M. Korte, V. Staiger, C. Gravel, P. Carroll, T. Bonhoeffer, and H. Thoenen    77

Neurotrophins in Cortical Development D.R. Riddle, A.K. McAllister, D.C. Lo, and L.C. Katz    85

In Vivo Development of Neuronal Structure and Function H. T. Cline, G.-Y. Wu, and R. Malinow    95

Cortical Dynamics and Visual Perception C.D. Gilbert, A. Das, M.K. Ito, M.K. Kapadia, and G. Westheimer    105

What Neurological Syndromes Can Tell Us about Human Nature: Some Lessons from Phantom Limbs, Capgras Syndrome, and Anosognosia V.S. Ramachandran    115



Sensory Perception

The Molecular Biology of Olfactory Perception P. Mombaerts, F. Wang, C. Dulac, R. Vassar, S.K. Chao, A. Nemes, M. Mendelsohn, J. Edmondson, and R. Axel    135

Information Coding in the Mammalian Olfactory System L.B. Buck    147

Olfactory Recognition and Discrimination in Caenorhabditis elegans J.H. Chou, E.R. Troemel, P. Sengupta, H.A. Colbert, L. Tong, D.M. Tobin, K. Roayaie, J.G. Crump, N.D. Dwyer, and C.I. Bargmann    157

Genetic Approaches to Mammalian Olfaction R.R. Reed    165

Biochemical and Transgenic Analysis of Gustducin's Role in Bitter and Sweet Transduction G. T. Wong, L. Ruiz-Avila, D. Ming, K.S. Gannon, and R.F. Margolskee    173



Memory .

Memory, Memory Impairment, and the Medial Temporal Lobe L.R. Squire and S.M. Zola    185

Nueral Mechanisms of Declrative Memory H. Eichenbaum, J. Dusek, B. Young, and M. Bunsey    197

A Return to Genetic Dissection of Memory in Drosophila T. Tully, G. Bolwig, J. Christensen, J. Connolly, M. DelVecchio, J. DeZazzo, J. Dubnau, C. Jones, S. Pinto, M. Regulski, B. Svedberg, and K. Velinzon    207

CaMKII Function in the Nervous System Explored from a Genetic Perspective M. Mayford, M.E. Bach, and E.R. Kandel    219

Hippocampal CA1-region-restricted Knockout of NMDARl Gene Disrupts Synaptic Plasticity, Place Fields, and Spatial Learning S. Tonegawa, J.Z. Tsien, T.J. McHugh, P. Huerta, K.I. Blum, and M.A. Wilson    225

Genetic Analysis of Function and Dysfunction in the Central Nervous System A.J. Silva, N. Fedorov, J. Kogan, P. Frankland, J. Coblentz, R. Lundsten, E. Friedman, A. Smith, Y. Cho, and K. P. Giese    239

Emotional Memory: A Search for Sites of Plasticity G.J. Quirk, J.L. Armony, J.C. Repa, X.-F. Li, and J.E. LeDoux    247

Neurobiology of Memory for Emotional Events: Converging Evidence from Infrahuman and Human Studies L. Cahill    259



Rhythms

A Drosophila Circadian Clock M. Rosbash, R. Allada, M. Dembinska, W.Q. Guo, M. Le, S. Marrus, Z. Qian, J.E. Rutila, J. Yaglom, and H. Zeng    265

Molecular Anatomy of a Light-sensitive Circadian Pacemaker in Drosophila M. W. Young, K. Wager-Smith, L. Vosshall, L. Saez, and M.P. Myers    279

Rhythmic Transcription and Autoregulatory Loops: Nuclear Pacemaker CREM M. Lamas, E. Lalli, N.S. Foulkes, and P. Sassone-Corsi    285

Forward Genetic Approaches to Circadian Clocks in Mice D.P. King and J.S. Takahashi    295



Channels and Receptors

Confocal-microscopic Visualization of Membrane Addition during Synaptic Exocytosis at Presynaptic Active Zones of Hair Cells A.J. Hudspeth and N.P. Issa    303

Multiple Myosin Isozymes and Hair-cell Function P.G. Gillespie, T. Hasson, J.A. Garcia, and D.P. Corey     309

Molecular and Cellular Plasticity in Developing Epileptic Brain J.L. Noebels, M.L. Sutherland, W.K. Nahm, and E. DiPasquale    319

Glutamate Receptor Autoimmunity in Rasmussen's Encephalitis J.D. McNamara, M. Patel, X.-P. He, S. Janumpalli, and K.D. Whitney    327

Molecular and Genetic Insights into Ligand Binding and Signal Transduction at the Inhibitory Glycine Receptor P.R. Schofield, J.W. Lynch, S. Rajendra, K.D. Pierce, C.A. Handford, and P.H. Barry    333

Nicotinic Receptors and Brain Plasticity J.-P. Changeux, A. Bessis, J.-P. Bourgeois, P.-J. Corringer, A. Devillers-Thiery, J.-L. Eisele, M. Kerszberg, C. Lena, N. Le Novere, M. Picciotto, and M. Zoli     343

The Nicotinic Acetylcholine Receptor at the Neuromuscular Junction: Assembly and Tyrosine Phosphorylation Z.-Z. Wang, C. Fuhrer, S. Shtrom, J.E. Sugiyama, M.J. Ferns, and Z.W. Hall     363

The GluR2 Hypothesis: Ca++-permeable AMPA Receptors in Delayed Neurodegeneration M.V.L. Bennett, D.E. Pellegrini-Giampietro, J.A. Gorter, E. Aronica, J.A. Connor, and R.S. Zukin    373



Neuronal Survival and Signaling

Zinc Neurotoxicity May Contribute to Selective Neuronal Death following Transient Global Cerebral Ischemia D.W. Choi    385

A Signaling Endosome Hypothesis to Explain NGF Actions: Potential Implications for Neurodegeneration E.C. Beattie, J. Zhou, M.L. Grimes, N. W. Bunnett, C.L. Howe, and W.C. Mobley     389

Neurotrophins and Their p75 Receptor B.D. Carter, G. Dechant, J.M. Frade, C. Kaltschmidt, and Y.-A. Barde    407

Nitric Oxide, Cell Multiplication, and Cell Survival N. Peunova, B. Kuzin, I. Roberts, C. O'Kane, and G. Enikolopov     417

The Role of Neuregulin (ARIA) at the Neuromuscular Junction K.M. Rosen, A. W. Sandrock, Jr., A.D.J. Goodearl, J.A. Loeb, and G.D. Fischbach     427

The Receptor Tyrosine Kinase MuSK Is Required for Neuromuscular Junction Formation and Is a Functional Receptor for Agrin D.J. Glass, T.M. DeChiara, T.N. Stitt, P.S. DiStefano, D.M. Valenzuela, and G.D. Yancopoulos     435

GDNF Is Required for Kidney Development and Enteric Innervation J.G. Pichel, L. Shen, H.Z. Sheng, A.-C. Granholm, J. Drago, A. Grinberg, E.J. Lee, S.P. Huang, M. Saarma, B.J. Hoffer, H. Sariola, and H. Westphal    445

Neuregulins as Potential Drugs for Neurological Disorders M.A. Marchionni, C.J. Kirk, I.J. Isaacs, C.J. Hoban, N.K. Mahanthappa, E.S. Anton, C. Chen, F. Wason, D. Lawson, F.P. T. Hamers, P.D. Canoll, R. Reynolds, B. Cannella, D. Meun, W.F. Holt, W.D. Matthew, L.-E. Chen, W.H. Gispen, C.S. Raine, J.L. Salzer, and D.I. Gwynne     459



Prions

Molecular Biology and Genetics of Prion Diseases S.B. Prusiner    473

Prion Protein Structure and Scrapie Replication: Theoretical, Spectroscopic, and Genetic Investigations P. Bamborough, H. Wille, G.C. Telling, F. Yehiely, S.B. Prusiner, and F.E. Cohen    495

The Role of PrP in Pathogenesis of Experimental Scrapie C. Weissmann, M. Fischer, A. Raeber, H. Bueler, A. Sailer, D. Shmerling, T. Rulicke, S. Brandner, and A. Aguzzi     511

A New Variant of Creutzfeldt-Jakob Disease: Neuropathological and Clinical Features J.W. Ironside, K. Sutherland, J.E. Bell, L. McCardle, C. Barrie, K. Estebeiro, M. Zeidler, and R.C. Will     523

Abnormal Plasma Membrane Properties and Functions in Prion-infected Cell Lines S.J. DeArmond, Y. Qiu, K. Wong, R. Nixon, W. Hyun, S.B. Prusiner, and W.C. Mobley    531

Prions of Yeast, [PSI] and [URE3], as Models for Neurodegenerative Diseases R.B. Wickner, D.C. Masison, H.K. Edskes, and M.-L. Maddelein    541

Alzheimer's Disease The Role of Presenilin 1 in the Genetics of Alzheimer's Disease R.F. Clark, M. Hutton, C. Talbot, M. Wragg, C. Lendon, F. Busfield, S.W. Han, J. Perez-Tur, M. Adams, R. Fuldner, G. Roberts, E. Karran J.Hardy, and A. Goate    551

Two Homologous Genes Causing Early-onset Familial Alzheimer's Disease P.H. St. George-Hyslop, C. Levesque, L. Levesque, J. Rommens, D. Westaway, and P.E. Fraser    559

Molecular Dissection of the Neurofibrillary Lesions of Alzheimer's Disease M. Goedert, M.G. Spillantini, M. Hasegawa, R. Jakes, R.A. Crowther, and A. Klug    565

Neuronal Sorting and Processing of Amyloid Precursor Protein: Implications for Alzheimer's Disease P.J. Tienari, B. De Strooper, E. Ikon en, N. Ida, M. Simons, C.L. Masters C.G. Dotti, and K. Beyreuther    575

Cell Biology of the b-Amyloid Precursor Protein and the Genetics of Alzheimer's Disease D.J. Selkoe    587

Protein:Protein Interactions in Alzheimer's Disease and the CAG Triplet Repeat Diseases W.J. Strittmatter, J.R. Burke, V.S. DeSerrano, D.Y. Huang, W. Matthew, A.M. Saunders, B.L. Scott, J.M. Vance, K.H. Weisgraber, and A.D. Roses    597



Neurodegenerative Diseases

Myotonic Dystrophy: Discussion of Molecular Mechanism C.T. Caskey, M.S. Swanson, and L. T. Timchenko    607

Huntington's Disease J.F. Gusella, S. McNeil, F. Persichetti, J. Srinidhi, A. Novelletto, E. Bird, P. Faber, J.-P. Vonsattel, R.H. Myers, and M.E. MacDonald    615

Targeted Inactivation of the Mouse Huntington's Disease Gene Homolog Hdh M.E. MacDonald, M. Duyao, T. Calzonetti, A. Auerbach, A. Ryan, G. Barnes,J.K. White, W. Auerbach, J.-P. Vonsattel, J.F. Gusella, and A.L. Joyner    627

Polyglutamine Expansions and Neurodegenerative Diseases F. Saudou, D. Devys, Y. Trottier, G. 1mbert, M.-E. Stoeckel, A. Brice, and J.-L. Mandel    639

Toward Understanding Polyglutamine-induced Neurological Disease in Spinocerebellar Ataxia Type 1 H.T. Orr and H.Y. Zoghbi    649

Molecular Mechanisms for Charcot-Marie- Tooth Disease and Related Demyelinating Peripheral Neuropathies L.E. Warner, L.T. Reiter, T. Murakami, and J.R. Lupski    659

Connexin32 and X-linked Charcot-Marie- Tooth Disease K.H. Fischbeck, S.M. Deschenes, L.J. Bone, and S.S. Scherer    673

The Molecular Basis of Fragile X Syndrome D.E. Eberhart and S.T. Warren    679

Molecular Characterization of the Protein Products of the Fragile X Syndrome Gene and the Survival of Motor Neurons Gene Q. Liu, H. Siomi, M.C. Siomi, U. Fischer, Y. Zhang, L. Wan, and G. Dreyfuss    689

ALS: Molecular Clues to the Jigsaw Puzzle of Neuronal Degeneration T. Siddique    699

Neurofilaments, Radial Growth of Axons, and Mechanisms of Motor Neuron Disease T.L. Williamson, J.R. Marszalek, J.D. Vechio, L.I. Bruijn, M.K. Lee, Z. Xu, R.H. Brown, Jr., and D.W. Cleveland    709

Neurodegenerative Diseases and Model Systems D.L. Price, D.R. Borchelt, P.C. Wong, C.A. Pardo, G. Thinakaran, M.K. Lee, D.W. Cleveland, and S.S. Sisodia    725

Extracellular Proteases in Neuronal Function and Degeneration S. Strickland, A. Gualandris, A.D. Rogove, and S.E. Tsirka    739

Molecular Analysis of Neurotransmitter Transport into Secretory Vesicles Y. Liu, C. Waites, D. Krantz, P. Tan, and R.H. Edwards    741



Dementia, Addiction, and Psychiatric Diseases

AIDS Dementia Complex: A Complex, Slow Virus "Model" of Acquired Genetic Neurodegenerative Disease R.W. Price    759

Quantitative Trait Loci Mapping in Mouse Models of Complex Behavior T.N. Ferraro and W.H. Berrettini    771

Recent Developments in the Genetics of Bipolar Disorder J.R. DePaulo, Jr. and F.J. McMahon    783

The Serotonin Transporter Gene and Affective Disorder A.J. Harmar, A.D. Ogilvie, S. Battersby, C.A.D. Smith, D.H.R. Blackwood, W.J. Muir, G. Fink, and G.M. Goodwin    791

The Johns Hopkins University Collaborative Schizophrenia Study: An Epidemiologic- Genetic Approach to Test the Heterogeneity Hypothesis and Identify Schizophrenia Susceptibility Genes A.E. Pulver, P.S. Wolyniec, D. Housman, H.H. Kazazian, S.E. Antonarakis, G. Nestadt, V.K. Lasseter, J.A. McGrath, B. Dombroski, M. Karayiorgou, C. Ton, J.-L. Blouin, and L. Kempf    791

Molecular Genetic Studies of Schizophrenia P. McGuffin and M.J. Owen    815

Support for Schizophrenia Vulnerability Loci on Chromosomes 6p and 8p from Irish Families R.E. Straub, C.J. MacLean, D. Walsh, and K.S. Kendler    823

Genotype and Phenotype Analysis at the 22q11 Schizophrenia Susceptibility Locus M. Karayiorgou, J.A. Gogos, B.L. Galke, J.A. Jeffery, G. Nestadt, P.S. Wolyniec, S.E. ntonarakis, H.H. Kazazian, D.E. Housman, D.A. Driscoll, and A.E. Pulver    835

Searching for Susceptibility Genes in Schizophrenia by Genetic Linkage Analysis D.B. Wildenauer, J. Hallmayer, S.G. Schwab, M. Albus, G.N. Eckstein, P. Zill, S. Hönig, M. Strauss, M. Borrmann, D. Lichtermann, R.P. Ebstein, B. Lerer, N. Risch, and W. Maier    845

Summary: Function and Dysfunction of the Nervous System Z.W. Hall    851





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