Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets

Abstract: 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....

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Autores principales: Paz, Mariela, Barrantes, Francisco José
Formato: Artículo
Lenguaje:Inglés
Publicado: American Chemical Society 2019
Materias:
MIASTENIA GRAVIS
ENFERMEDADES AUTOINMUNES
ANTICUERPOS
RECEPTORES
SISTEMA NERVIOSO
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/9010
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139123456789-9010
record_format dspace
institution Universidad Católica Argentina
institution_str I-33
repository_str R-139
collection Repositorio Institucional de la Universidad Católica Argentina (UCA)
language Inglés
topic MIASTENIA GRAVIS
ENFERMEDADES AUTOINMUNES
ANTICUERPOS
RECEPTORES
SISTEMA NERVIOSO
spellingShingle MIASTENIA GRAVIS
ENFERMEDADES AUTOINMUNES
ANTICUERPOS
RECEPTORES
SISTEMA NERVIOSO
Paz, Mariela
Barrantes, Francisco José
Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
topic_facet MIASTENIA GRAVIS
ENFERMEDADES AUTOINMUNES
ANTICUERPOS
RECEPTORES
SISTEMA NERVIOSO
description Abstract: 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.
format Artículo
author Paz, Mariela
Barrantes, Francisco José
author_facet Paz, Mariela
Barrantes, Francisco José
author_sort Paz, Mariela
title Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
title_short Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
title_full Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
title_fullStr Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
title_full_unstemmed Autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
title_sort autoimmune attack of the neuromuscular junction in myasthenia gravis : nicotinic acetylcholine receptors and other targets
publisher American Chemical Society
publishDate 2019
url https://repositorio.uca.edu.ar/handle/123456789/9010
work_keys_str_mv AT pazmariela autoimmuneattackoftheneuromuscularjunctioninmyastheniagravisnicotinicacetylcholinereceptorsandothertargets
AT barrantesfranciscojose autoimmuneattackoftheneuromuscularjunctioninmyastheniagravisnicotinicacetylcholinereceptorsandothertargets
bdutipo_str Repositorios
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