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pathogenetic role of a fibrogenic cytokine","volume":"96","author":"P Bernasconi","year":"1995","journal-title":"J Clin Invest"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.jns.2007.09.043","article-title":"Connective tissue growth factor is overexpressed in muscles of human muscular dystrophy","volume":"267","author":"G Sun","year":"2008","journal-title":"J Neurol Sci"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1016\/S0960-8966(02)00071-8","article-title":"Fibrogenic cytokines and extent of fibrosis in muscle of dogs with X-linked golden retriever muscular dystrophy","volume":"12","author":"L Passerini","year":"2002","journal-title":"Neuromuscul Disord"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1172\/JCI119513","article-title":"Inhibitors of the proteasome reduce the accelerated proteolysis in atrophying rat skeletal muscles","volume":"100","author":"NE Tawa","year":"1997","journal-title":"J Clin Invest"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1007\/s004010000229","article-title":"Proteasome expression in the skeletal muscles of patients with muscular dystrophy","volume":"100","author":"T Kumamoto","year":"200","journal-title":"Acta Neuropathol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1016\/S0002-9440(10)63523-7","article-title":"Proteasome inhibitor (MG-132) treatment of mdx mice rescues the expression and membrane localization of dystrophin and dystrophin-associated proteins","volume":"163","author":"G Bonuccelli","year":"2003","journal-title":"Am J Pathol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1096\/fj.03-0610com","article-title":"Multiple types of skeletal muscle atrophy involve a common program of changes in gene expression","volume":"18","author":"SH Lecker","year":"2004","journal-title":"Faseb J"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1186\/1471-2474-12-185","article-title":"The proteasome inhibitor MG132 reduces immobilization-induced skeletal muscle atrophy in mice","volume":"12","author":"AZ Caron","year":"2011","journal-title":"Musculoskelet Disord"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"C577","DOI":"10.1152\/ajpcell.00434.2005","article-title":"Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment","volume":"290","author":"S Assereto","year":"2006","journal-title":"Am J Physiol - 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Nature Biotechnology","volume":"26","author":"Y Sato","year":"2008"},{"key":"ref37","unstructured":"Rasband W S, U. S. National Institutes of Health, Bethesda, Maryland, USA, Available: <ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/imagej.nih.gov\/ij\/\" xlink:type=\"simple\">http:\/\/imagej.nih.gov\/ij\/<\/ext-link>, 1997\u20132012.Accessed 2009 Oct 12."},{"key":"ref38","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1186\/1479-5876-6-35","article-title":"Early transplantation of human immature dental pulp stem cells from baby teeth to golden retriever muscular dystrophy (GRMD) dogs: Local or systemic?","volume":"6","author":"I Kerkis","year":"2008","journal-title":"J Translat Med"},{"key":"ref39","first-page":"188","article-title":"Physical therapy assessment tools to evaluate disease progression and phenotype variability in Golden Retriever muscular dystrophy.Res Vet Sci","volume":"91","author":"TP Gaiad","year":"2011"},{"key":"ref40","unstructured":"Chen, CR. Handling, Restraint and Physical Examination of Dogs and Cats. Washington State University. Available: <ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/www.vetmed.wsu.edu\/resources\/restraint\/index.aspx\" xlink:type=\"simple\">http:\/\/www.vetmed.wsu.edu\/resources\/restraint\/index.aspx<\/ext-link>. 1999. Accessed 2011 Jan 11."},{"key":"ref41","first-page":"673","article-title":"Proteasome inhibition measurements: Clinical application, Clin Chem","volume":"46","author":"ES Lightcap","year":"2000"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"170","DOI":"10.2353\/ajpath.2008.071146","article-title":"Inhibition of proteasome activity promotes the correct localization of disease-causing alpha-sarcoglycan mutants in HEK-293 cells constitutively expressing beta-, gamma-, and delta-sarcoglycan","volume":"173","author":"S Gastaldello","year":"2008","journal-title":"Am J Pathol"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"255","DOI":"10.5326\/0380255","article-title":"Dystrophin-deficient muscular dystrophy in a labrador retriever","volume":"38","author":"RL Bergman","year":"2002","journal-title":"J Am Anim Hosp Assoc"},{"key":"ref44","unstructured":"Lipton B (1979) Muscle Regeneration. In: Mauro A, ed. New York, Raven Press, pp. 31\u201340."},{"key":"ref45","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/0022-510X(94)90050-7","article-title":"A role for mast-cells in the progression of duchenne muscular-dystrophy - correlations in dystrophin-deficient humans, dogs, and mice","volume":"122","author":"JRM Gorospe","year":"1994","journal-title":"J Neurol Sci"},{"key":"ref46","first-page":"687","article-title":"The muscular dystrophies, Lancet","volume":"359","author":"AEH Emery","year":"2002"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1002\/ana.410230511","article-title":"Monoclonal-antibody analysis of mononuclear-cells in myopathies .5. identification and quantitation of T8+ cyto-toxic and T8+ suppressor cells","volume":"23","author":"K Arahata","year":"1988","journal-title":"Ann Neurol"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1038\/labinvest.3780092","article-title":"T-cell-dependent fibrosis in the mdx dystrophic mouse","volume":"80","author":"J Morrison","year":"2000","journal-title":"Lab Invest"},{"key":"ref49","unstructured":"Cullen MJ, Mastaglia FL (1982) Pathological reactions of skeletal muscle. In: Livingstone C. ed. New York, pp. 88\u2013139."},{"key":"ref50","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1007\/BF00688053","article-title":"Progressive muscular-dystrophy in a golden retriever dog - light-microscope and ultrastructural features at 4 and 8 months","volume":"71","author":"BA Valentine","year":"1986","journal-title":"Acta Neuropathol"},{"key":"ref51","first-page":"553","article-title":"Regulation of the calpain and ubiquitin-proteasome systems in a canine model of muscular dystrophy.Muscle Nerve","volume":"44","author":"KM Wadosky","year":"2011"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"4804","DOI":"10.1200\/JCO.2004.12.185","article-title":"Phase I study of the proteasome inhibitor bortezomib in pediatric patients with refractory solid tumors: A children&apos;s oncology group study (ADVL0015)","volume":"22","author":"SM Blaney","year":"2004","journal-title":"J Clin Oncol"},{"key":"ref53","first-page":"2615","article-title":"Proteasome inhibitors: A novel class of potent and effective antitumor agents","volume":"59","author":"J Adams","year":"1999","journal-title":"Cancer Res"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1161\/CIRCRESAHA.107.152959","article-title":"Chronic proteasome inhibition contributes to coronary atherosclerosis","volume":"101","author":"J Herrmann","year":"2007","journal-title":"Circulation Res"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1111\/j.1939-1676.2001.tb02317.x","article-title":"Muscular dystrophy in female dogs","volume":"15","author":"GD Shelton","year":"2001","journal-title":"J Vet Intern Med"},{"key":"ref56","first-page":"1","article-title":"Canine x-linked muscular-dystrophy - morphological lesions, J Neurol Sci","volume":"97","author":"BA Valentine","year":"1990"},{"key":"ref57","first-page":"285","article-title":"IKK beta\/NF-kappa B activation causes severe muscle wasting in mice, Cell","volume":"119","author":"DS Cai","year":"2004"},{"key":"ref58","first-page":"889","article-title":"Interplay of IKK\/NF-kappa B signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy, J Clin Invest","volume":"117","author":"S Acharyya","year":"2007"},{"key":"ref59","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1038\/gt.2010.110","article-title":"Inhibition of the IKK\/NF-\u03baB pathway by AAV gene transfer improves muscle regeneration in older mdx mice","volume":"17","author":"Y Tang","year":"2010","journal-title":"Gene Ther"},{"key":"ref60","first-page":"1863","article-title":"Therapeutic Potential of Protepsome Inhibition in Duchenne and Becker Muscular Dystrophies, Am J Pathol","volume":"176","author":"E Gazzerro","year":"2010"},{"key":"ref61","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1038\/284470a0","article-title":"A role for collagen in the pathogenesis of muscular-dystrophy","volume":"284","author":"VC Duance","year":"1980","journal-title":"Nature"},{"key":"ref62","doi-asserted-by":"crossref","unstructured":"Gaiad TP, Araujo KPC, Caromano F, Ambrosio CE (2012) Duchenne Muscular Dystrophy: Experimental models on Physical Therapy. In: Hegde M, Ankala A, ed.. Muscular Dystrophy. Rijeka, Croatia: InTech; 2012. pp. 535\u2013544. <comment>doi:<ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"http:\/\/dx.doi.org\/10.5772\/30329\" xlink:type=\"simple\">10.5772\/30329<\/ext-link><\/comment>","DOI":"10.5772\/30329"},{"key":"ref63","first-page":"e44438","article-title":"Dystrophin deficiency compromises force production of the extensor carpi ulnaris muscle in the canine model of duchenne muscular dystrophy. 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