ePoster · Neuroscience
Myelinating Schwann cell alterations in the sciatic nerve of dystrophic mdx mice suggest a potential impact on the sensory-motor control of dystrophic muscles
Silvia di Nuzzo, Virginia Biddau, Valerio Magnaghi, Veronica Bonalume, Valentina Ferretti, Ada Maria Tata, Maria Egle De Stefano · FENS Forum 2024
Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disease characterized by the absence of the cytoskeletal protein dystrophin (Dp427). Short isoforms, such as Dp116 in myelinating Schwann cells (SCs), exist in specific cell types. In SCs, Dp116 binds to the dystrophin-associated glycoprotein complex (DGC), which crucially contributes to myelin integrity and fiber conduction properties. Our prior research on the sciatic nerve (SN) of wild-type and mdx mice, a DMD animal model lacking Dp427, revealed a significant decrease in all myelin protein levels, indicating compromised myelin