Microtubule dynamics in the peripheral nervous system : a matter of balanceMicrotubule dynamics in the peripheral nervous system : a matter of balance
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
VIB DMG - Peripheral Neuropathies Group
1(2011):6, p. 267-270
University of Antwerp
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.