The expansion of 300 CTG repeats in myotonic dystrophy transgenic mice does not induce sensory or motor neuropathy.

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Serval ID
serval:BIB_6F686EF685D9
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
The expansion of 300 CTG repeats in myotonic dystrophy transgenic mice does not induce sensory or motor neuropathy.
Journal
Acta neuropathologica
Author(s)
Gantelet E., Kraftsik R., Delaloye S., Gourdon G., Kuntzer T., Barakat-Walter I.
ISSN
0001-6322
Publication state
Published
Issued date
2007
Peer-reviewed
Oui
Volume
114
Number
2
Pages
175-85
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't - Publication Status: ppublish
Abstract
Although many studies have been carried out to verify the involvement of the peripheral nervous system (PNS) in dystrophia myotonica (DM1) patients, the results remain controversial. The generation of DM1 transgenic mice displaying the human DM1 phenotype provides a useful tool to investigate the type and incidence of structural abnormalities in the PNS. In the present study, the morphological and morphometric analysis of semi-thin sections of sciatic and sural nerves, lumbar dorsal root ganglia (DRG) and lumbar spinal cords revealed that in DM1 transgenic mice carrying 300 CTG repeats, there is no change in the number and diameter of myelinated axons compared to wild type. Only a non-significant reduction in the percentage of thin myelinated axons was detected in electron micrographs of ultra-thin sciatic nerve sections. Analysis of the number of neurons did not reveal a loss in number of either sensory neurons in the lumbar DRG or motor neurons in the lumbar spinal cord in these DM1 mice. Furthermore, in hind limb muscle sections, stained with a neurofilament antibody and alpha-bungarotoxin, the intramuscular axon arborization appeared normal in DM1 mice and undistinguishable from that in wild-type mice. Moreover, in DM1 mice, there was no irregularity in the structure or an increase in the endplate area. Also statistical analysis did not show an increase in endplate density or in the concentration of acetylcholine receptors. Altogether, these results suggest that 300 CTG repeats are not sufficient to induce axonopathy, demyelination or neuronopathies in this transgenic mouse model.
Keywords
Animals, Axons, Demyelinating Diseases, Ganglia, Spinal, Humans, Mice, Mice, Transgenic, Microscopy, Electron, Transmission, Motor Neurons, Myotonic Dystrophy, Neuromuscular Junction, Neurons, Afferent, Peripheral Nervous System Diseases, Sciatic Nerve, Spinal Cord, Sural Nerve, Trinucleotide Repeat Expansion
Pubmed
Web of science
Open Access
Yes
Create date
25/01/2008 12:44
Last modification date
01/10/2019 6:18
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