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Neuropathol Appl Neurobiol. 2010 Apr;36(2):133-56. doi: 10.1111/j.1365-2990.2010.01061.x. Epub 2010 Feb 19.

Review: neuromuscular synaptic vulnerability in motor neurone disease: amyotrophic lateral sclerosis and spinal muscular atrophy.

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  • 1Centre for Integrative Physiology, University of Edinburgh Medical School, Edinburgh, EH8 9XD, UK.

Abstract

Amid the great diversity of neurodegenerative conditions, there is a growing body of evidence that non-somatic (that is, synaptic and distal axonal) compartments of neurones are early and important subcellular sites of pathological change. In this review we discuss experimental data from human patients, animal models and in vitro systems showing that neuromuscular synapses are targeted in different forms of motor neurone disease (MND), including amyotrophic lateral sclerosis and spinal muscular atrophy. We highlight important developments revealing the heterogeneous nature of vulnerability in populations of lower motor units in MND and examine how progress in our understanding of the molecular pathways underlying MND may provide insights into the regulation of synaptic vulnerability and pathology. We conclude that future experiments developing therapeutic approaches specifically targeting neuromuscular synaptic vulnerability are likely to be required to prevent or delay disease onset and progression in human MND patients.

PMID:
20202121
[PubMed - indexed for MEDLINE]
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