Improvement of motor conduction velocity in hereditary neuropathy of LAMA2-CMD dy2J/dy2J mouse model by glatiramer acetate

•dy2J/dy2J mice peripheral neuropathy was confirmed by showing significantly slower NCV than WT.•Reduced dy2J/dy2J motor NCV improved significantly after GA-treatment.•The study suggests therapeutic effect for GA in LAMA2-CMD hereditary peripheral neuropathy. Glatiramer acetate (GA), an agent modulating the immune system, has been shown to cause significantly improved mobility and hind limb muscle strength in the dy2J/dy2J 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 dy2J/dy2J motor nerve conduction electrophysiologically. Left sciatic-tibial motor nerve conduction studies were performed on wild type (WT) mice (n = 10), control dy2J/dy2J mice (n = 11), and GA treated dy2J/dy2J mice (n = 10) at 18 weeks of age. Control dy2J/dy2J mice average velocities (34.49 ± 2.15 m/s) were significantly slower than WT (62.57 ± 2.23 m/s; p