DMD – BIOMARKERS & OUTCOME MEASURES: P.122 Translational implications of increased S100-beta and Tau5 proteins in dystrophic nerves of mdx mouse and rat models for Duchenne muscular dystrophy

Abstract

In dystrophic mdx mouse and dog models of Duchenne muscular dystrophy (DMD) repeated intrinsic muscle necrosis also damages the neuromuscular junctions (NMJs) with progressively altered NMJ morphology. We propose that these altered NMJs result in premature changes in dystrophic nerves and this was tested by immunoblotting. These analyses were built upon our observations related to normal ageing and sarcopenia, where time course studies show denervation of NMJs and myofibres along with neurodegeneration of peripheral nerves by 18 to 22 months(m), and also changes in normal old spinal cords.