Autoimmune Attack of the Neuromuscular Junction in Myasthenia Gravis: Nicotinic Acetylcholine Receptors and Other Targets
| Autor: | Mariela L. Paz, Francisco J. Barrantes |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Physiology
Cognitive Neuroscience Neuromuscular Junction SISTEMA NERVIOSO RECEPTORES Receptors Nicotinic Biochemistry Synaptic Transmission ENFERMEDADES AUTOINMUNES Neuromuscular junction 03 medical and health sciences 0302 clinical medicine Postsynaptic potential NEUROMUSCULAR JUNCTION Myasthenia Gravis medicine MYASTHENIA GRAVIS Animals Humans Cholinergic synapse Receptor 030304 developmental biology Acetylcholine receptor Autoantibodies 0303 health sciences NICOTINIC ACETYLCHOLINE RECEPTOR business.industry END PLATE Cell Biology General Medicine purl.org/becyt/ford/3.1 [https] medicine.disease Myasthenia gravis MIASTENIA GRAVIS Nicotinic acetylcholine receptor ANTIRECEPTOR ANTIBODY medicine.anatomical_structure Nicotinic agonist AUTOIMMUNE DISEASES ANTICUERPOS purl.org/becyt/ford/3 [https] business Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | CONICET Digital (CONICET) Consejo Nacional de Investigaciones Científicas y Técnicas instacron:CONICET Postprint del artículo publicado en ACS Chemical Neuroscience vol. 10, no. 5, 2019 Repositorio Institucional (UCA) Pontificia Universidad Católica Argentina instacron:UCA |
| ISSN: | 1948-7193 |
| Popis: | The nicotinic acetylcholine receptor (nAChR) family, the archetype member of the pentameric ligand-gated ion channels, is ubiquitously distributed in the central and peripheral nervous systems and its members are the targets for both genetic and acquired forms of neurological disorders. In the central nervous system nAChRs contribute to the pathological mechanisms of neurodegenerative disorders, such as Alzheimer and Parkinson diseases. In the peripheral nerve-muscle synapse, the vertebrate neuromuscular junction, ?classical? myasthenia gravis (MG) and other forms of neuromuscular transmission disorders are antibody-mediated autoimmune diseases. In MG, antibodies to the nAChR bind to the postsynaptic receptors and activate the classical complement pathway culminating in the formation of the membrane attack complex, with the subsequent destruction of the postsynaptic apparatus. Divalent nAChR-antibodies also cause internalization and loss of the nAChRs. Loss of receptors by either mechanism results in the muscle weakness and fatigability that typify the clinical manifestations of the disease. Other targets for antibodies, in a minority of patients, include muscle specific kinase (MuSK) and low-density lipoprotein related protein 4 (LRP4). This brief review analyzes the current status of muscle-type nAChR in relation to the pathogenesis of autoimmune diseases affecting the peripheral cholinergic synapse. Fil: Paz, Mariela Laura. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Estudios de la Inmunidad Humoral Prof. Ricardo A. Margni. Universidad de Buenos Aires. Facultad de Farmacia y Bioquímica. Instituto de Estudios de la Inmunidad Humoral Prof. Ricardo A. Margni; Argentina Fil: Barrantes, Francisco Jose. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Biomédicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Biomédicas; Argentina |
| Databáze: | OpenAIRE |
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