New movements in neurofilament transport, turnover and disease.

Publication Type:

Journal Article

Authors:

Barry, Devin M; Millecamps, Stéphanie; J-P Julien; Garcia, Michael L

Source:

Exp Cell Res, Volume 313, Issue 10, p.2110-20 (2007)

Keywords:

Animals, Axonal Transport, Axons, Charcot-Marie-Tooth Disease, Genetic Predisposition to Disease, Humans, Intermediate Filaments, Mutation, Nervous System, Neurofilament Proteins, Protein Subunits

Abstract:

<p>Revealing the mechanisms by which neurofilament transport and turnover are regulated has proven difficult over the years but recent studies have given new insight into these processes. Mature neurofilament fibers may incorporate a fourth functional subunit, alpha-internexin, as new evidence suggests. Recent findings have made the role of phosphorylation in regulating neurofilament transport velocity controversial. Kinesin and dynein may transport neurofilaments in slow axonal transport as they have been found to associate with neurofilaments. Neurofilament transport and turnover rates may be reduced depending on the existing stationary neurofilament network. Finally, mutations in neurofilament light that have been linked to Charcot-Marie-Tooth disease as well as other neurofilament abnormalities in human disease are discussed.</p>