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Pt. I Structure and Function of the Neuromuscular Junction
Structural Factors Influencing the Efficacy of Neuromuscular Transmission 1
Cell-to-Cell Signaling at the Neuromuscular Junction: The Dynamic Role of the Extracellular Matrix 13
The Role of Glial Cells in the Formation and Maintenance of the Neuromuscular Junction 19
Pt. II Structure and Function of the Acetylcholine Receptor
Myasthenia Gravis and the Tops and Bottoms of AChRs: Antigenic Structure of the MIR and Specific Immunosuppression of EAMG Using AChR Cytoplasmic Domains 29
Functional Organization and Conformational Dynamics of the Nicotinic Receptor: A Plausible Structural Interpretation of Myasthenic Mutations 42
Recent Structural and Mechanistic Insights into Endplate Acetylcholine Receptors 53
Peptide-Toxin Tools for Probing the Expression and Function of Fetal and Adult Subtypes of the Nicotinic Acetylcholine Receptor 61
Pt. III Muscle-Specific Kinase and Myasthenia Gravis
Clinical Phenotype of Muscle-specific Tyrosine Kinase-antibody-positive Myasthenia Gravis 71
Response to Therapy in Myasthenia Gravis with Anti-MuSK Antibodies 76
Myasthenia Gravis Seronegative for Acetylcholine Receptor Antibodies 84
Myasthenia Gravis Experimentally Induced with Muscle-specific Kinase 93
Pt. IV Congenital Myasthenic Syndromes
Congenital Myasthenic Syndromes and the Formation of the Neuromuscular Junction 99
Further Observations in Congenital Myasthenic Syndromes 1047
Pt. V Immunology of Autoimmune Disorders Involving Neuronal and Glial Channel Proteins
Anti-ganglioside Antibodies and the Presynaptic Motor Nerve Terminal 114
Neuronal Acetylcholine Receptor Autoimmunity124
Lambert-Eaton Myasthenic Syndrome: Tumor versus Nontumor Forms 129
Pt. VI Autoinunune Pathogenesis of Myasthenia Gravis: Current Concepts
Regulatory and Pathogenic Mechanisms in Human Autoimmune Myasthenia Gravis 135
Common Cellular and Diverse Genetic Basis of Thymoma-associated Myasthenia Gravis: Role of MHC Class II and AIRE Genes and Genetic Polymorphisms 143
How Promiscuity Promotes Tolerance: The Case of Myasthenia Gravis 157
Autoimmunizing Mechanisms in Thymoma and Thymus 163
Treatment of Myasthenia Gravis by Preventing Acetylcholine Receptor Modulation 174
Genetic Factors in Autoimmune Myasthenia Gravis 180
CD4+ T Cells and Cytokines in the Pathogenesis of Acquired Myasthenia Gravis 193
Classical Complement Pathway in Experimental Autoimmune Myasthenia Gravis Pathogenesis 210
Extraocular Muscle Susceptibility to Myasthenia Gravis: Unique Immunological Environment? 220
Pt. VII Clinical Trials of Myasthenia Gravis
Treatment and Clinical Research in Myasthenia Gravis: How Far Have We Come? 225
Rules of Evidence for Clinical Trials: The Science of Finding the Truth? 233
Myasthenia Gravis in the Elderly: Is It Different? 238
Immunosuppression of EAMG by IVIG Is Mediated by a Disease-specific Anti-immunoglobulin Fraction 244
Lessons from Two Trials of Mycophenolate Mofetil in Myasthenia Gravis 249
Tacrolimus for Myasthenia Gravis: A Clinical Study of 212 Patients 254
IVIG Treatment for Myasthenia Gravis: Effectiveness, Limitations, and Novel Therapeutic Strategies 264
Treatment of Myasthenia Gravis Acute Exacerbations with Intravenous Immunoglobulin 271
Pt. VIII Treatments on the Horizon for Myasthenia Gravis
Strategies for Treating Autoimmunity: Novel Insights from Experimental Myasthenia Gravis 276
Antisense Treatment for Myasthenia Gravis: Experience with Monarsen 283
Antigen-specific Apheresis of Pathogenic Autoantibodies from Myasthenia Gravis Sera 291
Etanercept Treatment in Myasthenia Gravis 300
Rebooting the Immune System with High-Dose Cyclophosphamide for Treatment of Refractory Myasthenia Gravis 305
Pt. IX Panel Discussion: Thymectomy for Myasthenia Gravis
Thymectomy for Nonthymomatous Myasthenia Gravis: A Critical Analysis 315
Thoracoscopic Thymectomy with the da Vinci Robotic System for Myasthenia Gravis 329
Extended Transcervical Thymectomy in the Treatment of Myasthenia Gravis 336
Status of the Thymectomy Trial for Nonthymomatous Myasthenia Gravis Patients Receiving Prednisone 344
List of Abstracts 348
Erratum for Ann. N.Y Acad. Sci. 1010: 441-445. Apoptosis: From Signaling Pathways to Therapeutic Tools, Marc Diederich, Ed. 387
Erratum for Ann. N.Y Acad. Sci. 1107: 308-318. Autoimmunity, Part C: The Mosaic of Autoimmunity, Yehuda Shoenfeld and M. Eric Gershwin, Eds. 388
Erratum for Ann. N.Y Acad. Sci. 1112: 140-153. Thymosins in Health and Disease, Allan L. Goldstein and Enrico Garaci, Eds 389
Erratum for Ann. NY Acad. Sci. 1113: 304-310. Stress Responses in Biology and Medicine: Stress of Life in Molecules, Cells, Organisms, and Psychosocial Communities, Peter Csermely, Tamas Korcsmaros, and Katalin Sulyok, Eds 390
Index of Contributors 391
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