New movements in CMT neurofilament transport, turnover and disease

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Exp Cell Res. 2007 Mar 21

New movements in neurofilament transport, turnover and disease.

Barry DM, Millecamps S, n JP, ML.

Division of Biological Sciences, University of Missouri-Columbia,

1201 East Rollins Street, Columbia, MO 65211, USA.

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.