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Bortezomib (Ps-341) Treatment Decreases Inflammation And Partially Rescues The Expression Of The Dystrophin-Glycoprotein Complex In Grmd Dogs., Karla P C Araujo, Gloria Bonuccelli, Caio N Duarte, Thais P Gaiad, Dayson F Moreira, David Feder, José E Belizario, Maria A Miglino, Michael P Lisanti, Carlos E Ambrosio

Department of Stem Cell Biology and Regenerative Medicine Faculty Papers & Presentations

Golden retriever muscular dystrophy (GRMD) is a genetic myopathy corresponding to Duchenne muscular dystrophy (DMD) in humans. Muscle atrophy is known to be associated with degradation of the dystrophin-glycoprotein complex (DGC) via the ubiquitin-proteasome pathway. In the present study, we investigated the effect of bortezomib treatment on the muscle fibers of GRMD dogs. Five GRMD dogs were examined; two were treated (TD- Treated dogs) with the proteasome inhibitor bortezomib, and three were control dogs (CD). Dogs were treated with bortezomib using the same treatment regimen used for multiple myeloma. Pharmacodynamics were evaluated by measuring the inhibition of 20S proteasome activity …