Measure of neuromuscular junction function and fiber morphometry

  • Measurement of electrophysiological parameters such as SNCV (sensory nerve conduction velocity) and CMAP (compound muscle action potential) are useful tools to assess the neuromuscular junction function. Used in combination with fiber morphometry measurement, these measurements can bring you a complete overview of the neuroprotective and neuro-regenerative effect of your compounds. An example of the relevance of this measurement is provided in the publication below.

  • Animal models



    Charcot-Marie-Tooth disease type 1A (CMT1A) is the most common inherited sensory and motor peripheralneuropathy. It is caused by PMP22 overexpression which leads to defects of peripheral myelination, loss of longaxons, and progressive impairment then disability. There is no treatment available despite observations thatmonotherapeutic interventions slow progression in rodent models (...)
    In vivo efficacy of the combinationwas tested in two models: CMT1A transgenic rats, and mice that recover from a nerve crush injury, a model toassess neuroprotection and regeneration. Combination of (RS)-baclofen, naltrexone hydrochloride and D-sorbitol,termed PXT3003, improved myelination in the Pmp22 transgenic co-culture cellular model, and moderatelydown-regulated Pmp22 mRNA expression in Schwannoma cells. In both in vitro systems, the combination of drugswas revealed to possess synergistic effects, which provided the rationale for in vivo clinical testing of rodent models.In Pmp22 transgenic CMT1A rats, PXT3003 down-regulated the Pmp22 to Mpz mRNA ratio, improved myelination ofsmall fibres, increased nerve conduction and ameliorated the clinical phenotype.