This review presents an overview of DMD and the pathological, immunological and molecular studies that have involved the GRMD model, highlighting its contribution to a better understanding pathophysiology and morphofunctional expression of the disease.
GRMD is characterized by progressive muscle weakness and atrophy.
The canine GRMD model is undoubtedly the closest to the human disease, presenting severe cardiac and skeletal alterations similar to those observed in those affected by DMD.
Bortezomib (PS-341) treatment decreases inflammation and partially rescues the expression of the dystrophinglycoprotein complex in GRMD dogs.
Effects of an immunosuppressive treatment in the GRMD dog model of Duchenne muscular dystrophy.