| Introduction |
|
xiii | |
|
|
|
Part I. Structure and Function of the Neuromuscular Junction: Physiology and Neurotransmitter Release |
|
|
|
Neurophysiology of the Neuromuscular Junction: Overview |
|
|
1 | (10) |
|
|
|
Ca2+ Channels and Synaptic Transmission at the Adult, Neonatal, and P/Q-Type Deficient Neuromuscular Junction |
|
|
11 | (7) |
|
|
|
|
|
|
|
|
|
Erika S. Piedras Renteria |
|
|
|
|
Effect of Inherited Abnormalities of Calcium Regulation on Human Neuromuscular Transmission |
|
|
18 | (11) |
|
|
|
|
|
|
|
Transmitter Release Deficits at the Neuromuscular Synapse of Mice with Mutations in the Cav2.1 (1A) Subunit of the P/Q-Type Ca2+ Channel |
|
|
29 | (4) |
|
|
|
A. M. Van Den Maagdenberg |
|
|
|
|
|
|
|
|
Protein Ubiquitylation and Synaptic Function |
|
|
33 | (8) |
|
|
|
|
|
|
|
Part II. Structure and Function of the Neuromuscular Junction: Structure and Function of the Acetylcholine Receptor |
|
|
|
Nicotinic Acetylcholine Receptors of Muscles and Nerves: Comparison of Their Structures, Functional Roles and Vulnerability to Pathology |
|
|
41 | (12) |
|
|
|
Synapse-Specific Gene Expression at the Neuromuscular Junction |
|
|
53 | (13) |
|
|
|
|
|
|
|
|
|
Regulation of Nicotinic Acetylcholine Receptor Assembly |
|
|
66 | (15) |
|
|
|
|
|
|
|
Structure and Function of AChBP, Homologue of the Ligand-Binding Domain of the Nicotinic Acetylcholine Receptor |
|
|
81 | (12) |
|
|
|
|
|
|
|
|
|
The Binding Site of Acetylcholine Receptor: From Synthetic Peptides to Solution and Crystal Structure |
|
|
93 | (8) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Ephraim Katchalski-Katzir |
|
|
Agonist-Induced Transitions of the Acetylcholine Receptor |
|
|
101 | (13) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Part III. Structure and Function of the Neuromuscular Junction: Genetic Biochemical Disorders |
|
|
|
Structural Abnormalities of the AChR Caused by Mutations Underlying Congenital Myasthenic Syndromes |
|
|
114 | (11) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Congenital Myasthenic Syndrome in Cattle due to Homozygosity for a Truncating Mutation in the Acetylcholine Receptor (AChR) Epsilon-Subunit Gene |
|
|
125 | (3) |
|
|
|
|
|
|
|
|
|
Mechanistic Diversity Underlying Fast Channel Congenital Myasthenic Syndromes |
|
|
128 | (10) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Congenital Myasthenic Syndromes: Multiple Molecular Targets at the Neuromuscular Junction |
|
|
138 | (23) |
|
|
|
|
|
|
|
|
|
Part IV. Autoimmunity: General Concepts |
|
|
|
Autoimmune Diseases as the Loss of Active ``Self-Control'' |
|
|
161 | (17) |
|
|
|
Immunology of Paraneoplastic Syndromes: Overview |
|
|
178 | (9) |
|
|
|
Part V. Immunology of Autoimmune Disorders Involving the Neuron and Presynaptic Nerve Terminal |
|
|
|
Pathogenic Autoantibodies in the Lambert-Eaton Myasthenic Syndrome |
|
|
187 | (9) |
|
|
|
|
|
|
|
|
|
|
|
LEMS IgG Binds to Extracellular Determinants on N-Type Voltage-Gated Calcium Channels, but Does Not Reduce VGCC Expression |
|
|
196 | (4) |
|
|
|
|
|
|
|
|
|
HLA-B8 in Patients with the Lambert-Eaton Myasthenic Syndrome Reduces Likelihood of Associated Small Cell Lung Carcinoma |
|
|
200 | (2) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Autoimmune Disorders of Neuronal Potassium Channels |
|
|
202 | (9) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Neuronal Ganglionic Acetylcholine Receptor Autoimmunity |
|
|
211 | (4) |
|
|
|
|
|
Humoral and Cellular Autoimmune Responses in Stiff Person Syndrome |
|
|
215 | (8) |
|
|
|
|
|
|
|
|
|
Part VI. Immunology of Postsynaptic Autoimmune Disorders: The Thymus |
|
|
|
The Role of Thymomas in the Development of Myasthenia Gravis |
|
|
223 | (14) |
|
|
|
Hans Konrad Muller-Hermelink |
|
|
|
|
Scenarios for Autoimmunization of T and B Cells in Myasthenia Gravis |
|
|
237 | (20) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
A New Model Linking Intrathymic Acetylcholine Receptor Expression and the Pathogenesis of Myasthenia Gravis |
|
|
257 | (9) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Human Myoid Cells Protect Thymocytes from Apoptosis |
|
|
266 | (4) |
|
|
|
|
|
Analysis of SjTREC Levels in Thymus from MG Patients and Normal Children |
|
|
270 | (5) |
|
|
|
|
|
|
|
|
|
|
|
Analysis of CD4+CD25+ Cell Population in the Thymus from MG Patients |
|
|
275 | (3) |
|
|
|
|
|
|
|
|
|
Expression of Transforming Growth Factor-1 in Thymus of Myasthenia Gravis Patients: Correlation with Pathological Abnormalities |
|
|
278 | (6) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Part VII. Immunology of Postsynaptic Autoimmune Disorders: T Cells and Immunoregulation |
|
|
|
T Cells and Cytokines in the Pathogenesis of Acquired Myasthenia Gravis |
|
|
284 | (24) |
|
|
|
|
|
|
|
|
|
Immunoregulation in Experimental Autoimmune Myasthenia Gravis---about T Cells, Antibodies and Endplates |
|
|
308 | (10) |
|
|
|
|
|
|
|
|
|
|
|
Circulating CD4+CD25+ and CD4+CD25- T Cells in Myasthenia Gravis |
|
|
318 | (2) |
|
|
|
|
|
|
|
|
|
|
|
Rationale for a T Cell Receptor Peptide Therapy in Myasthenia Gravis |
|
|
320 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Part VIII. Immunology of Postsynaptic Autoimmune Disorders: Antigenic Targets and Human Disease |
|
|
|
Antibodies in Myasthenia Gravis and Related Disorders |
|
|
324 | (12) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Differences between the ε and γ Subunits of the Acetylcholine Receptor (AChR) May Be Significant in Autoimmune Myasthenia Gravis |
|
|
336 | (3) |
|
|
|
|
|
Use of Peptide: HLA Class II Complexes to Study Specific T Cells in Autoimmune Myasthenia Gravis |
|
|
339 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Pathogenesis of Myositis and Myasthenia Associated with Titin and Ryanodine Receptor Antibodies |
|
|
343 | (8) |
|
|
|
|
|
|
|
|
|
|
|
Antibodies in Sera of Patients with Late-Onset Myasthenia Gravis Recognize the PEVK Domain of Titin |
|
|
351 | (5) |
|
|
|
|
|
Jennifer Butter Worth-Robinette |
|
|
|
|
|
|
|
|
Identification of Disease-Specific Autoantibodies in Seronegative Myasthenia Gravis |
|
|
356 | (3) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Muscle and Neuronal Autoantibody Markers of Thymoma: Neurological Correlations |
|
|
359 | (3) |
|
|
|
|
|
Susceptibility of Ocular Tissues to Autoimmune Diseases |
|
|
362 | (13) |
|
|
|
|
|
|
|
|
|
|
|
Part IX. Immunology of Postsynaptic Autoimmune Disorders: Antigenic Targets and Experimental Myasthenia |
|
|
|
Induction of Myasthenia Gravis in HLA Transgenic Mice by Immunization with Human Acetylcholine Receptors |
|
|
375 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Production and Characterization of a T Cell Receptor Transgenic Mouse Recognizing the Immunodominant Epitope of the Torpedo californica Acetylcholine Receptor |
|
|
379 | (5) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Epitope Repertoire of Th1 and Th2 Cells Reactive with the Mouse Muscle AChR α Subunit in C57B1/6 Mice |
|
|
384 | (4) |
|
|
|
|
|
|
|
|
|
|
|
Epitope Spreading to Hidden Cytoplasmic Regions of the Acetylcholine Receptor in Experimental Autoimmune Myasthenia Gravis |
|
|
388 | (3) |
|
|
|
|
|
|
|
|
|
Immunization with Rat-, but Not Torpedo-Derived 97--116 Peptide of the AChR α-Subunit Induces Experimental Myasthenia Gravis (EAMG) in Lewis Rat |
|
|
391 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Effect on T Cell Recognition and Immunogenicity of Alanine-Substituted Peptides Corresponding to 97--116 Sequence of the Rat AChR α-Subunit |
|
|
395 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Characterization of a Fully Human IgG1 Reconstructed from an Anti-AChR Fab |
|
|
399 | (2) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Roles of Complex Gangliosides at the Neuromuscular Junction |
|
|
401 | (3) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Role of Munc 18-1 in Synaptic Plasticity at the Myasthenic Neuromuscular Junction |
|
|
404 | (3) |
|
|
|
|
|
|
|
Part X. Epidemiology and Measurements in Myasthenia Gravis |
|
|
|
The Epidemiology of Myasthenia Gravis |
|
|
407 | (6) |
|
|
|
Myasthenia Gravis (MG): Epidemiological Data and Prognostic Factors |
|
|
413 | (11) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Myasthenia Gravis in Individuals over Forty |
|
|
424 | (8) |
|
|
|
|
|
|
|
|
|
Standards of Measurements in Myasthenia Gravis |
|
|
432 | (8) |
|
|
|
A Simple Manual Muscle Test for Myasthenia Gravis: Validation and Comparison with the QMG Score |
|
|
440 | (5) |
|
|
|
Bernadette Tucker-Lipscomb |
|
|
|
|
Standards of Measurements in Myasthenia Gravis |
|
|
445 | (8) |
|
|
|
|
|
|
|
Part XI. Current Diagnosis and Treatment: Pre- and Postsynaptic Immune Syndromes |
|
|
|
Three Forms of Immune Myasthenia |
|
|
453 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Treatment Principles in the Management of Autoimmune Myasthenia Gravis |
|
|
457 | (16) |
|
|
|
|
|
Development of a Thymectomy Trial in Nonthymomatous Myasthenia Gravis Patients Receiving Immunosuppressive Therapy |
|
|
473 | (8) |
|
|
|
|
|
|
|
|
|
|
|
Thymectomy and Antimuscle Antibodies in Nonthymomatous Myasthenia Gravis |
|
|
481 | (10) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Prognostic Factors of Thymectomy in Patients with Myasthenia Gravis: A Cohort of 132 Patients |
|
|
491 | (3) |
|
Jose Francisco Tellez-Zenteno |
|
|
|
|
|
|
|
|
|
|
Mycophenolate Mofetil for Myasthenia Gravis: A Double-Blind, Placebo-Controlled, Pilot Study |
|
|
494 | (6) |
|
|
|
|
|
|
|
|
|
Lambert-Eaton Myasthenic Syndrome: Diagnosis and Treatment |
|
|
500 | (9) |
|
|
|
Abnormal Single-Fiber Electromyography in Patients Not Having Myasthenia: Risk for Diagnostic Confusion? |
|
|
509 | (3) |
|
|
|
Part XII. Future Therapies in Myasthenia |
|
|
|
Treatment of Autoimmune Disease by Adoptive Cellular Gene Therapy |
|
|
512 | (8) |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
Specific Immunotherapy of Experimental Myasthenia by Genetically Engineered APCs: The ``Guided Missile'' Strategy |
|
|
520 | (13) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Suppression of Myasthenia Gravis by Antigen-Specific Mucosal Tolerance and Modulation of Cytokines and Costimulatory Factors |
|
|
533 | (4) |
|
|
|
|
|
|
|
|
|
|
|
|
|
Suppression of EAMG in Lewis Rats by IL-10-Exposed Dendritic Cells |
|
|
537 | (2) |
|
|
|
|
|
|
|
Future Therapeutic Strategies in Autoimmune Myasthenia Gravis |
|
|
539 | (10) |
|
|
|
|
|
|
| Index of Contributors |
|
549 | |
