Molecular and cellular pathways of neurodegeneration in motor neurone disease.

P J Shaw

Journal: Journal of neurology, neurosurgery, and psychiatry 2005;76(8):1046-57

PMID: 16024877

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Abstract

The process of neuronal degeneration in motor neurone disease is complex. Several genetic alterations may be involved in motor neurone injury in familial amyotrophic lateral sclerosis, less is known about the genetic and environmental factors involved in the commoner sporadic form of the disease. Most is known about the mechanisms of motor neurone degeneration in the subtype of disease caused by SOD1 mutations, but even here there appears to be a complex interplay between multiple pathogenic processes including oxidative stress, protein aggregation, mitochondrial dysfunction excitotoxicity, and impaired axonal transport. There is new evidence that non-neuronal cells in the vicinity of motor neurones may contribute to neuronal injury. The final demise of motor neurones is likely to involve a programmed cell death pathway resembling apoptosis.

Address: [email protected]

DOI: 10.1136/jnnp.2004.048652

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MeSH Terms: Amyotrophic Lateral Sclerosis, Axonal Transport, Biological Transport, Calcium-Binding Proteins, Cell Aggregation, Cell Death, Chromosomes, Human, Pair 9, DNA Helicases, Dynactin Complex, Environment, Exons, Humans, Kv Channel-Interacting Proteins, Microtubule-Associated Proteins, Mitochondrial Myopathies, Motor Neuron Disease, Multifunctional Enzymes, Nerve Degeneration, Neural Pathways, Oxidative Stress, Point Mutation, RNA Helicases, Risk Factors, Superoxide Dismutase, Superoxide Dismutase-1