Immune-mediated mechanisms potentially regulate the disease time-course of duchenne muscular dystrophy and provide targets for therapeutic intervention
NP Evans, SA Misyak, JL Robertson… - PM&R, 2009 - Elsevier
Duchenne muscular dystrophy is a lethal muscle-wasting disease that affects boys.
Mutations in the dystrophin gene result in the absence of the dystrophin glycoprotein
complex (DGC) from muscle plasma membranes. In healthy muscle fibers, the DGC forms a
link between the extracellular matrix and the cytoskeleton to protect against contraction-
induced membrane lesions and to regulate cell signaling. The absence of the DGC results in
aberrant regulation of inflammatory signaling cascades. Inflammation is a key pathological …
Mutations in the dystrophin gene result in the absence of the dystrophin glycoprotein
complex (DGC) from muscle plasma membranes. In healthy muscle fibers, the DGC forms a
link between the extracellular matrix and the cytoskeleton to protect against contraction-
induced membrane lesions and to regulate cell signaling. The absence of the DGC results in
aberrant regulation of inflammatory signaling cascades. Inflammation is a key pathological …