Compartmental neurodegeneration and synaptic plasticity in the Wld(s) mutant mouse

This review focuses on recent developments in our understanding of neurodegeneration at the mammalian neuromuscular junction. We provide evidence to support a hypothesis of compartmental neurodegeneration, whereby synaptic degeneration occurs by a separate, distinct mechanism from cell body and axon...

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Bibliographic Details
Published in:The Journal of physiology 2001-08, Vol.534 (Pt 3), p.627-639
Main Authors: Gillingwater, T H, Ribchester, R R
Format: Article
Language:English
Subjects:
Animals
Mice
Mice, Mutant Strains - physiology
Neuronal Plasticity - physiology
Synapses - physiology
Wallerian Degeneration - genetics
Wallerian Degeneration - physiopathology
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Summary:This review focuses on recent developments in our understanding of neurodegeneration at the mammalian neuromuscular junction. We provide evidence to support a hypothesis of compartmental neurodegeneration, whereby synaptic degeneration occurs by a separate, distinct mechanism from cell body and axonal degeneration. Studies of the spontaneous mutant Wld(s) mouse, in which Wallerian degeneration is characteristically slow, provide key evidence in support of this hypothesis. Some features of synaptic degeneration in the absence of Wallerian degeneration resemble synapse elimination in neonatal muscle. This and other forms of synaptic plasticity may be accessible to further investigations, exploiting advantages afforded by the Wld(s) mutant, or transgenic mice that express the Wld(s) gene.
ISSN:0022-3751
DOI:10.1111/j.1469-7793.2001.00627.x