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Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy

In the peripheral nervous system disorders plasticity is related to changes on the axon and Schwann cell biology, and the synaptic formations and connections, which could be also a focus for therapeutic research. Charcot-Marie-Tooth disease (CMT) represents a large group of inherited peripheral neur...

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Detalles Bibliográficos
Autores principales: Juárez, Paula, Palau, Francesc
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3382403/
https://www.ncbi.nlm.nih.gov/pubmed/22745917
http://dx.doi.org/10.1155/2012/171636
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author Juárez, Paula
Palau, Francesc
author_facet Juárez, Paula
Palau, Francesc
author_sort Juárez, Paula
collection PubMed
description In the peripheral nervous system disorders plasticity is related to changes on the axon and Schwann cell biology, and the synaptic formations and connections, which could be also a focus for therapeutic research. Charcot-Marie-Tooth disease (CMT) represents a large group of inherited peripheral neuropathies that involve mainly both motor and sensory nerves and induce muscular atrophy and weakness. Genetic analysis has identified several pathways and molecular mechanisms involving myelin structure and proper nerve myelination, transcriptional regulation, protein turnover, vesicle trafficking, axonal transport and mitochondrial dynamics. These pathogenic mechanisms affect the continuous signaling and dialogue between the Schwann cell and the axon, having as final result the loss of myelin and nerve maintenance; however, some late onset axonal CMT neuropathies are a consequence of Schwann cell specific changes not affecting myelin. Comprehension of molecular pathways involved in Schwann cell-axonal interactions is likely not only to increase the understanding of nerve biology but also to identify the molecular targets and cell pathways to design novel therapeutic approaches for inherited neuropathies but also for most common peripheral neuropathies. These approaches should improve the plasticity of the synaptic connections at the neuromuscular junction and regenerate cell viability based on improving myelin and axon interaction.
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spelling pubmed-33824032012-06-28 Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy Juárez, Paula Palau, Francesc Neural Plast Review Article In the peripheral nervous system disorders plasticity is related to changes on the axon and Schwann cell biology, and the synaptic formations and connections, which could be also a focus for therapeutic research. Charcot-Marie-Tooth disease (CMT) represents a large group of inherited peripheral neuropathies that involve mainly both motor and sensory nerves and induce muscular atrophy and weakness. Genetic analysis has identified several pathways and molecular mechanisms involving myelin structure and proper nerve myelination, transcriptional regulation, protein turnover, vesicle trafficking, axonal transport and mitochondrial dynamics. These pathogenic mechanisms affect the continuous signaling and dialogue between the Schwann cell and the axon, having as final result the loss of myelin and nerve maintenance; however, some late onset axonal CMT neuropathies are a consequence of Schwann cell specific changes not affecting myelin. Comprehension of molecular pathways involved in Schwann cell-axonal interactions is likely not only to increase the understanding of nerve biology but also to identify the molecular targets and cell pathways to design novel therapeutic approaches for inherited neuropathies but also for most common peripheral neuropathies. These approaches should improve the plasticity of the synaptic connections at the neuromuscular junction and regenerate cell viability based on improving myelin and axon interaction. Hindawi Publishing Corporation 2012 2012-06-13 /pmc/articles/PMC3382403/ /pubmed/22745917 http://dx.doi.org/10.1155/2012/171636 Text en Copyright © 2012 P. Juárez and F. Palau. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Juárez, Paula
Palau, Francesc
Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title_full Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title_fullStr Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title_full_unstemmed Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title_short Neural and Molecular Features on Charcot-Marie-Tooth Disease Plasticity and Therapy
title_sort neural and molecular features on charcot-marie-tooth disease plasticity and therapy
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3382403/
https://www.ncbi.nlm.nih.gov/pubmed/22745917
http://dx.doi.org/10.1155/2012/171636
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