RESEARCH: Role of axonal transport in neuro-degenerative disorders

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Motor neurons rely on motor proteins.

Holzbaur EL.

University of Pennsylvania, D400 s Building,

3700 Hamilton Walk, Philadelphia, PA 19104-6085, USA.

The importance of active axonal transport to the

neuron has been highlighted by the recent discoveries

that mutations in microtubule motor proteins result in

neurodegenerative diseases. Mutations affecting

microtubule motor function have been shown to cause

hereditary forms of Charcot-Marie-Tooth disease (type

2A), hereditary spastic paraplegia and motor neuron

disease. Although motor neurons appear to be uniquely

susceptible to defects in axonal transport, recent

work has identified links between perturbations in

axonal transport and the pathogenesis of other

neurodegenerative diseases such as Huntington's

disease and Alzheimer's disease. More broadly,

cytoskeletal abnormalities might also be at the root

of related disorders such as spinal muscular atrophy,

supporting a key role for axonal transport in the

pathogenesis of many neurodegenerative diseases.

SOURCE: Trends Cell Biol. 2004 May;14(5):233-40.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=1\

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