Motor neurons rely on motor proteins (2A)

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

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.