Cargando…

Microtubule dynamics in the peripheral nervous system: A matter of balance

The special architecture of neurons in the peripheral nervous system, with axons extending for long distances, represents a major challenge for the intracellular transport system. Two recent studies show that mutations in the small heat shock protein HSPB1, which cause an axonal type of Charcot-Mari...

Descripción completa

Detalles Bibliográficos
Autores principales: Almeida-Souza, Leonardo, Timmerman, Vincent, Janssens, Sophie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Landes Bioscience 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3337128/
https://www.ncbi.nlm.nih.gov/pubmed/22545178
http://dx.doi.org/10.4161/bioa.1.6.19198
_version_ 1782231035572912128
author Almeida-Souza, Leonardo
Timmerman, Vincent
Janssens, Sophie
author_facet Almeida-Souza, Leonardo
Timmerman, Vincent
Janssens, Sophie
author_sort Almeida-Souza, Leonardo
collection PubMed
description The special architecture of neurons in the peripheral nervous system, with axons extending for long distances, represents a major challenge for the intracellular transport system. Two recent studies show that mutations in the small heat shock protein HSPB1, which cause an axonal type of Charcot-Marie-Tooth (CMT) neuropathy, affect microtubule dynamics and impede axonal transport. Intriguingly, while at presymptomatic age the neurons in the mutant HSPB1 mouse show a hyperstable microtubule network, at postsymptomatic age, the microtubule network completely lost its stability as reflected by a marked decrease in tubulin acetylation levels. We here propose a model explaining the role of microtubule stabilization and tubulin acetylation in the pathogenesis of HSPB1 mutations.
format Online
Article
Text
id pubmed-3337128
institution National Center for Biotechnology Information
language English
publishDate 2011
publisher Landes Bioscience
record_format MEDLINE/PubMed
spelling pubmed-33371282012-05-07 Microtubule dynamics in the peripheral nervous system: A matter of balance Almeida-Souza, Leonardo Timmerman, Vincent Janssens, Sophie Bioarchitecture Perspective The special architecture of neurons in the peripheral nervous system, with axons extending for long distances, represents a major challenge for the intracellular transport system. Two recent studies show that mutations in the small heat shock protein HSPB1, which cause an axonal type of Charcot-Marie-Tooth (CMT) neuropathy, affect microtubule dynamics and impede axonal transport. Intriguingly, while at presymptomatic age the neurons in the mutant HSPB1 mouse show a hyperstable microtubule network, at postsymptomatic age, the microtubule network completely lost its stability as reflected by a marked decrease in tubulin acetylation levels. We here propose a model explaining the role of microtubule stabilization and tubulin acetylation in the pathogenesis of HSPB1 mutations. Landes Bioscience 2011-11-01 /pmc/articles/PMC3337128/ /pubmed/22545178 http://dx.doi.org/10.4161/bioa.1.6.19198 Text en Copyright © 2011 Landes Bioscience http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited.
spellingShingle Perspective
Almeida-Souza, Leonardo
Timmerman, Vincent
Janssens, Sophie
Microtubule dynamics in the peripheral nervous system: A matter of balance
title Microtubule dynamics in the peripheral nervous system: A matter of balance
title_full Microtubule dynamics in the peripheral nervous system: A matter of balance
title_fullStr Microtubule dynamics in the peripheral nervous system: A matter of balance
title_full_unstemmed Microtubule dynamics in the peripheral nervous system: A matter of balance
title_short Microtubule dynamics in the peripheral nervous system: A matter of balance
title_sort microtubule dynamics in the peripheral nervous system: a matter of balance
topic Perspective
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3337128/
https://www.ncbi.nlm.nih.gov/pubmed/22545178
http://dx.doi.org/10.4161/bioa.1.6.19198
work_keys_str_mv AT almeidasouzaleonardo microtubuledynamicsintheperipheralnervoussystemamatterofbalance
AT timmermanvincent microtubuledynamicsintheperipheralnervoussystemamatterofbalance
AT janssenssophie microtubuledynamicsintheperipheralnervoussystemamatterofbalance