title: Microtubule dynamics in the peripheral nervous system: A matter of balance.
abstract: 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.
authors.forename: Leonardo
authors.initials: L
authors.lastname: Almeida-Souza
authors.forename: Vincent
authors.initials: V
authors.lastname: Timmerman
authors.forename: Sophie
authors.initials: S
authors.lastname: Janssens