Defective neurofilament transport in mouse models of amyotrophic lateral sclerosis: a review.
Neurofilament proteins synthesized in the cell body of neurons are assembled and transported into axons, where they influence axon radial growth, axonal transport, and nerve conduction velocities. In diseased states, neurofilaments accumulate in cell bodies and proximal axons of affected neurons, and these lesions are characteristic of many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), spinal muscular atrophy (SMA), Charcot-Marie-Tooth disease type 2 (CMT2), and hereditary sensory motor neuropathy. Although the molecular mechanisms that contribute to these accumulations are not yet identified, transgenic mouse models are beginning to provide insight into the role of neurofilament transport in disease-related dysfunction of neurons. This review addresses axonal transport in mouse models of ALS and the special significance of neurofilament transport in this disease.
Rao
Mala V
MV
Center for Dementia Research, Nathan Kline Institute/Department of Psychiatry, NYU School of Medicine, 140 Old Orangeburg Road, Orangeburg, New York 10962, USA. rao@nki.rfmh.org
Nixon
Ralph A
RA
D000690:Amyotrophic Lateral Sclerosis / Q000209:etiology / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D001370:Axonal Transport; D007381:Intermediate Filament Proteins / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D016900:Neurofilament Proteins / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism; D010641:Phenotype; D013482:Superoxide Dismutase / Q000235:genetics; D000072105:Superoxide Dismutase-1
D007381:Intermediate Filament Proteins; D016900:Neurofilament Proteins; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1