Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis

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Abstract: Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles. The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction, the nicotinic ace...

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Autor principal: Barrantes, Francisco José
Formato: Artículo
Lenguaje:Inglés
Publicado: Elsevier 2021
Materias:
ENFERMEDADES AUTOINMUNES
MIASTENIA GRAVIS
DEBILIDAD MUSCULAR
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/11574
https://doi.org/10.4103/1673-5374.290880
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139-123456789-11574
record_format dspace
spelling I33-R139-123456789-115742023-11-22T21:48:13Z Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis Barrantes, Francisco José ENFERMEDADES AUTOINMUNES MIASTENIA GRAVIS DEBILIDAD MUSCULAR Abstract: Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles. The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction, the nicotinic acetylcholine receptor. Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse. This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis. The review also covers the application of a powerful biophysical technique, superresolution optical microscopy, to image the nicotinic receptor in live cells and follow its motional dynamics. The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor, as observed in myasthenia gravis. 2021-06-02T13:40:17Z 2021-06-02T13:40:17Z 2021 Artículo Barrantes F.J. Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis [en línea]. Neural Regeneration Research. 2021, 16(2). Disponible en: https://repositorio.uca.edu.ar/handle/123456789/11574 1673-5374 https://repositorio.uca.edu.ar/handle/123456789/11574 https://doi.org/10.4103/1673-5374.290880 32859770 eng Acceso abierto http://creativecommons.org/licenses/by-nc-sa/4.0/ application/pdf Elsevier Neural Regeneration Research Vol.16, No.2, 2021
institution Universidad Católica Argentina
institution_str I-33
repository_str R-139
collection Repositorio Institucional de la Universidad Católica Argentina (UCA)
language Inglés
topic ENFERMEDADES AUTOINMUNES
MIASTENIA GRAVIS
DEBILIDAD MUSCULAR
spellingShingle ENFERMEDADES AUTOINMUNES
MIASTENIA GRAVIS
DEBILIDAD MUSCULAR
Barrantes, Francisco José
Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
topic_facet ENFERMEDADES AUTOINMUNES
MIASTENIA GRAVIS
DEBILIDAD MUSCULAR
description Abstract: Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles. The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction, the nicotinic acetylcholine receptor. Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse. This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis. The review also covers the application of a powerful biophysical technique, superresolution optical microscopy, to image the nicotinic receptor in live cells and follow its motional dynamics. The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor, as observed in myasthenia gravis.
format Artículo
author Barrantes, Francisco José
author_facet Barrantes, Francisco José
author_sort Barrantes, Francisco José
title Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
title_short Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
title_full Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
title_fullStr Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
title_full_unstemmed Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
title_sort possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis
publisher Elsevier
publishDate 2021
url https://repositorio.uca.edu.ar/handle/123456789/11574
https://doi.org/10.4103/1673-5374.290880
work_keys_str_mv AT barrantesfranciscojose possibleimplicationsofdysregulatednicotinicacetylcholinereceptordiffusionandnanoclusterformationinmyastheniagravis
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