Improvement of motor conduction velocity in hereditary neuropathy of LAMA2-CMD dy 2J /dy 2J mouse model by glatiramer acetate

Glatiramer acetate (GA), an agent modulating the immune system, has been shown to cause significantly improved mobility and hind limb muscle strength in the dy /dy mouse model for LAMA2-congenital muscular dystrophy (LAMA2-CMD). In view of these findings and the prominent peripheral nervous system i...

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Veröffentlicht in:Clinical neurophysiology 2019-10, Vol.130 (10), p.1988
Hauptverfasser: Rabie, Malcolm, Yanay, Nurit, Fellig, Yakov, Konikov-Rozenman, Jenya, Nevo, Yoram
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Sprache:eng
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Zusammenfassung:Glatiramer acetate (GA), an agent modulating the immune system, has been shown to cause significantly improved mobility and hind limb muscle strength in the dy /dy mouse model for LAMA2-congenital muscular dystrophy (LAMA2-CMD). In view of these findings and the prominent peripheral nervous system involvement in this laminin-α2 disorder we evaluated GA's effect on dy /dy motor nerve conduction electrophysiologically. Left sciatic-tibial motor nerve conduction studies were performed on wild type (WT) mice (n = 10), control dy /dy mice (n = 11), and GA treated dy /dy mice (n = 10) at 18 weeks of age. Control dy /dy mice average velocities (34.49 ± 2.15 m/s) were significantly slower than WT (62.57 ± 2.23 m/s; p 
ISSN:1872-8952
DOI:10.1016/j.clinph.2019.07.029