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 |
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Hauptverfasser: | , , , , |
Format: | Artikel |
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 |
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ISSN: | 1872-8952 |
DOI: | 10.1016/j.clinph.2019.07.029 |