{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T08:31:28Z","timestamp":1783240288551,"version":"3.54.6"},"reference-count":161,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2020,6,28]],"date-time":"2020-06-28T00:00:00Z","timestamp":1593302400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007372","name":"Association Belge contre les Maladies Neuro-Musculaires","doi-asserted-by":"publisher","award":["2019-2020\/07"],"award-info":[{"award-number":["2019-2020\/07"]}],"id":[{"id":"10.13039\/100007372","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>In Duchenne muscular dystrophy (DMD), the activation of proinflammatory and metabolic cellular pathways in skeletal muscle cells is an inherent characteristic. Synthetic glucocorticoid intake counteracts the majority of these mechanisms. However, glucocorticoids induce burdensome secondary effects, including hypertension, arrhythmias, hyperglycemia, osteoporosis, weight gain, growth delay, skin thinning, cushingoid appearance, and tissue-specific glucocorticoid resistance. Hence, lowering the glucocorticoid dosage could be beneficial for DMD patients. A more profound insight into the major cellular pathways that are stabilized after synthetic glucocorticoid administration in DMD is needed when searching for the molecules able to achieve similar pathway stabilization. This review provides a concise overview of the major anti-inflammatory pathways, as well as the metabolic effects of glucocorticoids in the skeletal muscle affected in DMD. The known drugs able to stabilize these pathways, and which could potentially be combined with glucocorticoid therapy as steroid-sparing agents, are described. This could create new opportunities for testing in DMD animal models and\/or clinical trials, possibly leading to smaller glucocorticoids dosage regimens for DMD patients.<\/jats:p>","DOI":"10.3390\/ijms21134596","type":"journal-article","created":{"date-parts":[[2020,6,29]],"date-time":"2020-06-29T05:28:56Z","timestamp":1593408536000},"page":"4596","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Anti-Inflammatory and General Glucocorticoid Physiology in Skeletal Muscles Affected by Duchenne Muscular Dystrophy: Exploration of Steroid-Sparing Agents"],"prefix":"10.3390","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1911-0173","authenticated-orcid":false,"given":"Sandrine","family":"Herbelet","sequence":"first","affiliation":[{"name":"Department of Head and Skin, Division of Neurology, Ghent University and Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3084-2722","authenticated-orcid":false,"given":"Arthur","family":"Rodenbach","sequence":"additional","affiliation":[{"name":"Department of Head and Skin, Division of Neurology, Ghent University and Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9403-4401","authenticated-orcid":false,"given":"Boel","family":"De Paepe","sequence":"additional","affiliation":[{"name":"Department of Head and Skin, Division of Neurology, Ghent University and Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"},{"name":"Neuromuscular Reference Center, Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan L.","family":"De Bleecker","sequence":"additional","affiliation":[{"name":"Department of Head and Skin, Division of Neurology, Ghent University and Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"},{"name":"Neuromuscular Reference Center, Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,6,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1083\/jcb.201212142","article-title":"Cellular and molecular mechanisms underlying muscular dystrophy","volume":"201","author":"Rahimov","year":"2013","journal-title":"J. Cell Biol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/j.bbamem.2013.08.023","article-title":"Dystrophin complex functions as a scaffold for signalling proteins","volume":"1838","author":"Constantin","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1146\/annurev-genom-090314-025003","article-title":"The pathogenesis and therapy of muscular dystrophies","volume":"16","author":"Guiraud","year":"2015","journal-title":"Annu. Rev. Genom. Hum. Genet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.pediatrneurol.2006.09.016","article-title":"Pathophysiology of Duchenne muscular dystrophy: Current hypotheses","volume":"36","author":"Deconick","year":"2007","journal-title":"Pediatr. Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1177\/0192623317734823","article-title":"Duchenne and Becker muscular dystrophies: A review of animal models, clinical end points, and biomarker quantification","volume":"45","author":"Wilson","year":"2017","journal-title":"Toxic. Pathol."},{"key":"ref_6","first-page":"CD003725","article-title":"Corticoids for the treatment of Duchenne muscular dystrophy (review)","volume":"5","author":"Matthews","year":"2016","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1136\/jnnp-2012-303902","article-title":"Long-term benefits and adverse effects of intermittent versus daily glucocorticoids in boys with Duchenne muscular dystrophy","volume":"84","author":"Ricotti","year":"2013","journal-title":"J. Neurol. Neurosurg. Psychiatry."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1210\/er.2014-1010","article-title":"Comprehensive overview of the structure and regulation of the glucocorticoid receptor","volume":"35","author":"Vandevyver","year":"2014","journal-title":"Endocr. Rev."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1128\/MMBR.00064-15","article-title":"The interactome of the glucocorticoid receptor and its influence on the actions of glucocorticoids in combating inflammatory and infectious diseases","volume":"80","author":"Petta","year":"2016","journal-title":"Microbiol. Mol. Biol. Rev."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1136\/thorax.55.7.603","article-title":"Molecular mechanisms of glucocorticoid action: What is important?","volume":"55","author":"Newton","year":"2000","journal-title":"Thorax"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1569","DOI":"10.1002\/emmm.201302621","article-title":"VBP15, a novel anti-inflammatory and membrane-stabilizer, improves muscular dystrophy without side effects","volume":"5","author":"Heier","year":"2013","journal-title":"EMBO Mol. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"299rv4","DOI":"10.1126\/scitranslmed.aaa7322","article-title":"Immune-mediated pathology in Duchenne muscular dystrophy","volume":"7","author":"Rosenberg","year":"2015","journal-title":"Sci. Transl. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1016\/j.molmed.2007.09.002","article-title":"Signaling to NF-\u03baB by toll-like receptors","volume":"13","author":"Kawai","year":"2007","journal-title":"Trends Mol. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2604","DOI":"10.1093\/hmg\/ddt656","article-title":"Role of toll-like receptors in the pathogenesis of dystrophin-deficient skeletal and heart muscle","volume":"23","author":"Yu","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"508","DOI":"10.3389\/fphys.2017.00508","article-title":"Inhibition of toll-like receptor signaling as a promising therapy for inflammatory diseases: A journey from molecular to nano therapeutics","volume":"8","author":"Gao","year":"2017","journal-title":"Front. Physiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1096\/fj.04-1859fje","article-title":"Glucocorticoid treatment alleviates dystrophic myofiber pathology by activation of the calcineurin\/NF-AT pathway","volume":"18","author":"Chakkalakal","year":"2004","journal-title":"FASEB J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1002\/cpt.522","article-title":"Evidence of NI-0101 pharmacological activity, an anti-TLR4 antibody, in a randomized phase I dose escalation study in healthy volunteers receiving LPS","volume":"101","author":"Monnet","year":"2017","journal-title":"Clin. Pharmacol. Ther."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1186\/s13075-016-1215-7","article-title":"Molecular alterations in skeletal muscle in rheumatoid arthritis are related to disease activity, physical inactivity, and disability","volume":"19","author":"Huffman","year":"2017","journal-title":"Arthritis Res. Ther."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1016\/j.bbadis.2015.04.013","article-title":"Opposing roles of miR-21 and miR-29 in the progression of fibrosis in Duchenne muscular dystrophy","volume":"1852","author":"Zanotti","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1152\/physiolgenomics.00134.2017","article-title":"Muscle miRNAome shows suppression of chronic inflammatory miRNAs with both prednisone and vamorolone","volume":"50","author":"Fiorillo","year":"2018","journal-title":"Physiol. Genom."},{"key":"ref_21","first-page":"2","article-title":"The changed transcriptome of muscular dystrophy and inflammatory myopathy: Contributions of non-coding RNAs to muscle damage and recovery","volume":"3","year":"2019","journal-title":"OBM Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"28097","DOI":"10.1038\/srep28097","article-title":"Circulating miRNAs are generic and versatile therapeutic monitoring biomarkers in muscular dystrophies","volume":"6","author":"Israeli","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Brusa, R., Magri, F., Bresolin, N., Comi, G.P., and Corti, S. (2020). Noncoding RNAs in Duchenne and Becker muscular dystrophies: Role in pathogenesis and future prognostic and therapeutic perspectives. Cell. Mol. Life Sci., Available online: https:\/\/pubmed.ncbi.nlm.nih.gov\/32350552\/.","DOI":"10.1007\/s00018-020-03537-4"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.1016\/j.immuni.2013.06.004","article-title":"miR-142\u20143p prevents macrophage differentiation during cancer-induced myelopoiesis","volume":"38","author":"Sonda","year":"2013","journal-title":"Immunity"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"345","DOI":"10.7150\/ijbs.10921","article-title":"MiR-206, a key modulator of skeletal muscle development and disease","volume":"11","author":"Ma","year":"2015","journal-title":"Int. J. Biol. Sci."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.omtn.2018.05.011","article-title":"Micro-RNA- 206 downregulation improves therapeutic gene expression and motor function in mdx mice","volume":"12","author":"Bulaklak","year":"2018","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1002\/mus.23312","article-title":"Altered mitogen-activated protein kinase signaling in dystrophic (mdx) muscle","volume":"46","author":"Smythe","year":"2012","journal-title":"Muscle Nerve."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"633","DOI":"10.2353\/ajpath.2007.060344","article-title":"Modulation of p38 MAPK cascade and metalloproteinase activity in diaphragm muscle in response to free radical scavengers administration in dystrophin-deficient mdx mice","volume":"170","author":"Hnia","year":"2007","journal-title":"Am. J. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1016\/0009-8981(82)90140-1","article-title":"Carnitine metabolism in early stages of Duchenne muscular dystrophy","volume":"122","author":"Berthillier","year":"1982","journal-title":"Clin. Chim. Acta"},{"key":"ref_30","first-page":"97","article-title":"L-carnitine supplementation in duchenne muscular dystrophy steroid-naive patients: A pilot study","volume":"3","year":"2013","journal-title":"Curr. Top. Nutraceut. Res."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"5452","DOI":"10.1093\/hmg\/ddu270","article-title":"P38\u03b1 MAPK underlies muscular dystrophy and myofiber death through a Bax-dependent mechanism","volume":"23","author":"Wissing","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s00109-009-0550-4","article-title":"Therapeutic targeting of signaling pathways in muscular dystrophy","volume":"88","author":"Bhatnagar","year":"2010","journal-title":"J. Mol. Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1278","DOI":"10.1016\/S0960-9822(01)00397-9","article-title":"Activation of JNK1 contributes to dystrophic muscle pathogenesis","volume":"11","author":"Kolodziejczyk","year":"2001","journal-title":"Curr. Biol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.3389\/fimmu.2019.01176","article-title":"Direct inhibition of IRF-dependent transcriptional regulatory mechanisms associated with disease","volume":"10","author":"Antonczyk","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Aziz, N., Son, Y.-J., and Cho, J.Y. (2018). Thymoquinone suppresses IRF-3-mediated expression of type I interferons via suppression of TBK1. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19051355"},{"key":"ref_36","first-page":"16","article-title":"Activation of NF-\u03baB pathway in Duchenne muscular dystrophy: Relation to age","volume":"30","author":"Messina","year":"2011","journal-title":"Acta Myol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1016\/j.nbd.2011.05.008","article-title":"Systemic delivery of NEMO binding domain\/IKK\u03b3 inhibitory peptide to young mdx mice improves dystrophic skeletal muscle histopathology","volume":"43","author":"Reay","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"234","DOI":"10.1016\/j.expneurol.2005.11.021","article-title":"Nuclear factor kappa-B blockade reduces skeletal muscle degeneration and enhances muscle function in mdx mice","volume":"198","author":"Messina","year":"2006","journal-title":"Exp. Neurol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"3431","DOI":"10.1038\/s41467-018-05910-1","article-title":"NF-\u03baB inhibition rescues cardiac function by remodeling calcium genes in a Duchenne muscular dystrophy model","volume":"9","author":"Peterson","year":"2018","journal-title":"Nat. Comm."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1186\/1479-5876-9-68","article-title":"Improvement of cardiac contractile function by peptide-based inhibition of NF-B in the utrophin\/dystrophin-deficient murine model of muscular dystrophy","volume":"9","author":"Xu","year":"2011","journal-title":"J. Transl. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1172\/JCI30556","article-title":"Interplay of IKK\/NF-kappaB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy","volume":"117","author":"Acharyya","year":"2007","journal-title":"J. Clin. Investig."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"508","DOI":"10.2119\/molmed.2010.00263","article-title":"Peptide-based inhibition of NF-kappaB rescues diaphragm muscle contractile dysfunction in a murine model of Duchenne muscular dystrophy","volume":"17","author":"Peterson","year":"2011","journal-title":"Mol. Med."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Zhao, J., Zhang, L., Mu, X., Doebelin, C., Nguyen, W., Wallace, C., Reay, D.P., McGowan, S.J., Corbo, L., and Clemens, P.R. (2018). Development of novel NEMO-binding domain mimetics for inhibiting IKK\/NF-\u03baB activation. PLoS Biol., 16.","DOI":"10.1371\/journal.pbio.2004663"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"442","DOI":"10.2119\/molmed.2013.00141","article-title":"D-amino acid substitution of peptide-mediated NF-\u03baB suppression in mdx mice preserves therapeutic benefit in skeletal muscle, but causes kidney toxicity","volume":"21","author":"Reay","year":"2015","journal-title":"Mol. Med."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1002\/ar.21294","article-title":"The influence of passive stretch and NF-\u03baB inhibitors on the morphology of dystrophic (mdx) muscle fibers","volume":"294","author":"Siegel","year":"2011","journal-title":"Anat. Rec. Adv. Integr. Anat. Evol. Biol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1016\/j.nbd.2005.05.003","article-title":"Chronic treatment with agents that stabilize cytosolic IKB-A enhances survival and improves resting membrane potential in mdx muscle fibers subjected to chronic passive stretch","volume":"20","author":"Carlson","year":"2005","journal-title":"Neurobiol. Dis."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.nmd.2008.10.006","article-title":"Treatment with inhibitors of the NF-\u03baB pathway improves whole body tension development in the mdx mouse","volume":"19","author":"Siegel","year":"2009","journal-title":"Neuromuscl. Disord."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"918","DOI":"10.2353\/ajpath.2006.050673","article-title":"Lipid peroxidation inhibition blunts nuclear factor-\u03baB activation, reduces skeletal muscle degeneration, and enhances muscle function in mdx mice","volume":"168","author":"Messina","year":"2006","journal-title":"Am. J. Pathol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"2003","DOI":"10.1113\/jphysiol.2007.148338","article-title":"N-acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice","volume":"586","author":"Whitehead","year":"2008","journal-title":"J. Physiol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/j.nmd.2011.11.007","article-title":"N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis","volume":"22","author":"Terrill","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/j.clnu.2012.06.001","article-title":"N-Acetylcysteine treatment reduces TNF-\u03b1 levels and myonecrosis in diaphragm muscle of mdx mice","volume":"32","author":"Ferretti","year":"2013","journal-title":"Clin. Nutr."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"7093","DOI":"10.1113\/JP274229","article-title":"Pre-clinical evaluation of N-acetylcysteine reveals side effects in the mdx mouse model of Duchenne muscular dystrophy","volume":"595","author":"Pinniger","year":"2017","journal-title":"J. Physiol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"e90341","DOI":"10.1172\/jci.insight.90341","article-title":"Disease-modifying effects of orally bioavailable NF-\u03baB inhibitors in dystrophin-deficient mice","volume":"1","author":"Hammers","year":"2016","journal-title":"JCI Insight"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"43","DOI":"10.3233\/JND-180341","article-title":"Phase 1 study of edasalonexent (CAT-1004), an oral NF-\u03baB inhibitor, in pediatric patients with Duchenne muscular dystrophy","volume":"6","author":"Finanger","year":"2019","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1002\/jcph.842","article-title":"A novel NF-\u03baB inhibitor, Edasalonexent (CAT-1004), in development as a disease-modifying treatment for patients with Duchenne muscular dystrophy: Phase 1 safety, pharmacokinetics, and pharmacodynamics in adult subjects","volume":"57","author":"Donovan","year":"2017","journal-title":"J. Clin. Pharmacol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"S51.006","DOI":"10.1212\/WNL.92.15_supplement.S51.006","article-title":"Edasalonexent, an NF-\u03baB inhibitor, slows longer-term disease progression on multiple functional and MRI assessments compared to control period in 4 to 7-years old patients with Duchenne muscular dystrophy","volume":"92","author":"Finkel","year":"2019","journal-title":"Neurology"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/S0014-5793(03)00175-3","article-title":"Ets, AP-1 and GATA factor families regulate the utrophin B promoter: Potential regulatory mechanisms for endothelial-specific expression","volume":"538","author":"Perkins","year":"2003","journal-title":"FEBS Lett."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"248","DOI":"10.2353\/ajpath.2010.091176","article-title":"Matrix metalloproteinase inhibitor Batimastat alleviates pathology and improves skeletal muscle function in dystrophin-deficient mdx mice","volume":"177","author":"Kumar","year":"2010","journal-title":"Am. J. Pathol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s11010-008-9716-6","article-title":"Protein-DNA array-based identification of transcription factor activities differentially regulated in skeletal muscle of normal and dystrophin-deficient mdx mice","volume":"312","author":"Dogra","year":"2008","journal-title":"Mol. Cell Biochem."},{"key":"ref_60","doi-asserted-by":"crossref","unstructured":"Umansky, K.B., Gruenbaum-Cohen, Y., Tsoory, M., Feldmesser, E., Goldenberg, D., Brenner, O., and Groner, Y. (2015). Runx1 transcription factor is required for myoblasts proliferation during muscle regeneration. PLoS Genet., 11.","DOI":"10.1371\/journal.pgen.1005457"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1405","DOI":"10.1016\/S0891-5849(01)00719-5","article-title":"NF kappa B and AP-1 mediate transcriptional responses to oxidative stress in skeletal muscle cells","volume":"31","author":"Zhou","year":"2001","journal-title":"Free Radic. Biol. Med."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"e23282","DOI":"10.4161\/jkst.23282","article-title":"JAK-STAT pathway and myogenic differentiation","volume":"2","author":"Jang","year":"2013","journal-title":"JAK-STAT"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"500","DOI":"10.3389\/fphys.2019.00500","article-title":"The JAK\/STAT pathway in skeletal muscle pathophysiology","volume":"10","author":"Moresi","year":"2019","journal-title":"Front. Physiol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/s13395-017-0140-z","article-title":"Treatment with the anti-IL-6 receptor antibody attenuates muscular dystrophy via promoting skeletal muscle regeneration in dystrophin-\/utrophin-deficient mice","volume":"7","author":"Wada","year":"2017","journal-title":"Skelet. Muscle"},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Guadagnin, E., M\u00e1zala, D., and Chen, Y.-W. (2018). STAT3 in skeletal muscle function and disorders. Int. J. Mol Sci., 19.","DOI":"10.3390\/ijms19082265"},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Kostek, M.C., Nagaraju, K., Pistilli, E., Sali, A., Lai, S.H., Gordon, B., and Chen, Y.-W. (2012). IL-6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse. BMC Musculoskelet. Disord., 106.","DOI":"10.1186\/1471-2474-13-106"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1677\/joe.0.1590323","article-title":"Crosstalk between the interleukin-6 (IL-6)-JAK-STAT and the glucocorticoid-nuclear receptor pathway: Synergistic activation of IL-6 response element by IL-6 and glucocorticoid","volume":"159","author":"Takeda","year":"1998","journal-title":"J. Endocrinol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.bbadis.2011.01.002","article-title":"Activation of AKT signaling promotes cell growth and survival in \u03b17\u03b21 integrin-mediated alleviation of muscular dystrophy","volume":"1812","author":"Boppart","year":"2011","journal-title":"Biochim. Biophys. Acta- Mol. Basis Dis."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1038\/nrd.2017.267","article-title":"JAK inhibition as a therapeutic strategy for immune and inflammatory diseases","volume":"17","author":"Schwartz","year":"2017","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"999","DOI":"10.2353\/ajpath.2009.080537","article-title":"Valproic acid activates the PI3K\/Akt\/mTOR pathway in muscle and ameliorates pathology in a mouse model of Duchenne muscular dystrophy","volume":"174","author":"Gurpur","year":"2009","journal-title":"Am. J. Pathol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"C188","DOI":"10.1152\/ajpcell.00542.2005","article-title":"Regulation of myostatin expression and myoblast differentiation by FoxO and SMAD transcription factors","volume":"292","author":"Allen","year":"2007","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"209","DOI":"10.2217\/fnl.12.3","article-title":"TGF-\u03b2 signaling in Duchenne muscular dystrophy","volume":"7","author":"Kemaladewi","year":"2012","journal-title":"Future Neurol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1212\/01.wnl.0000173836.09176.c4","article-title":"Early onset of inflammation and later involvement of TGF\u03b2 in Duchenne muscular dystrophy","volume":"65","author":"Chen","year":"2005","journal-title":"Neurology"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/2044-5040-1-19","article-title":"Role of TGF-\u03b2 signaling in inherited and acquired myopathies","volume":"1","author":"Burks","year":"2011","journal-title":"Skelet. Muscle"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.3892\/etm.2017.4105","article-title":"Expression levels of TGF-\u03b21 and CTGF are associated with the severity of Duchenne muscular dystrophy","volume":"13","author":"Song","year":"2017","journal-title":"Exp. Ther. Med."},{"key":"ref_76","doi-asserted-by":"crossref","unstructured":"Ismaeel, A., Kim, J.-S., Kirk, J.S., Smith, R.S., Bohannon, W.T., and Koutakis, P. (2019). Role of transforming growth factor-\u03b2 in skeletal muscle fibrosis: A review. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20102446"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.coph.2017.04.002","article-title":"Pharmacological advances for treatment in Duchenne muscular dystrophy","volume":"34","author":"Guiraud","year":"2017","journal-title":"Curr. Opin. Pharmacol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.lfs.2015.10.023","article-title":"Chronic effects of losartan on the muscles and the serologic profiles of mdx mice","volume":"15","author":"Lee","year":"2015","journal-title":"Life Sci."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1237","DOI":"10.1038\/sj.ki.5000034","article-title":"Losartan improves resistance artery lesions and prevents CTGF and TGF-beta production in mild hypertensive patients","volume":"69","author":"Granados","year":"2006","journal-title":"Kidney Int."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"2611","DOI":"10.1016\/j.ajpath.2011.02.024","article-title":"Inhibiting TGF-\u03b2 activity improves respiratory function in mdx mice","volume":"178","author":"Nelson","year":"2011","journal-title":"Am. J. Pathol."},{"key":"ref_81","doi-asserted-by":"crossref","unstructured":"Bish, L.T., Yarchoan, M., Sleeper, M.M., Gazzara, J., and Morine, K.J. (2011). Chronic Losartan administration reduces mortality and preserves cardiac but not skeletal muscle function in dystrophic Mice. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0020856"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1016\/0959-8049(94)90544-4","article-title":"Suramin inhibits growth and transforming growth factor -\u03b21 (TGF-\u03b21) binding in osteosarcoma cell lines","volume":"30","author":"Kloen","year":"1994","journal-title":"Eur. J. Cancer"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1002\/mus.21869","article-title":"Prevention of muscle fibrosis and myonecrosis in mdx mice by suramin, a TGF-\u03b21 blocker","volume":"43","author":"Taniguti","year":"2010","journal-title":"Muscle Nerve"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1002\/mus.21944","article-title":"Soluble activin receptor type IIB increases forward pulling tension in the mdx mouse","volume":"43","author":"Carlson","year":"2011","journal-title":"Muscle Nerve"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1002\/mus.25268","article-title":"Myostatin inhibitor ACE-031 treatment of ambulatory boys with Duchenne muscular dystrophy: Results of a randomized, placebo-controlled clinical trial","volume":"55","author":"Campbell","year":"2017","journal-title":"Muscle Nerve"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1038\/mt.2009.322","article-title":"Combination of myostatin pathway interference and dystrophin rescue enhances tetanic and specific force in dystrophic mdx mice","volume":"18","author":"Dumonceaux","year":"2010","journal-title":"Mol. Ther."},{"key":"ref_87","first-page":"464","article-title":"TGF\u03b2 and BMP signaling in skeletal muscle: Potential significance for muscle-related disorders","volume":"25","author":"Sartori","year":"2014","journal-title":"Cell"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/j.nbd.2010.10.003","article-title":"BMP antagonists enhance myogenic differentiation and ameliorate the dystrophic phenotype in a DMD mouse model","volume":"41","author":"Shi","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1016\/j.ajpath.2012.10.018","article-title":"The anticancer drug tamoxifen counteracts the pathology in a mouse model of Duchenne muscular dystrophy","volume":"182","author":"Dorchies","year":"2013","journal-title":"Am. J. Pathol."},{"key":"ref_90","first-page":"2617","article-title":"Bmi1 enhances skeletal muscle regeneration through MT1-mediated oxidative stress protection in a mouse model of dystrophinopathy","volume":"13","author":"Zhang","year":"2014","journal-title":"J. Exp. Med."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"7741","DOI":"10.1073\/pnas.1802425115","article-title":"Monitoring disease activity noninvasively in the mdx model of Duchenne muscular dystrophy","volume":"115","author":"Filareto","year":"2018","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1186\/s13063-019-3740-6","article-title":"Tamoxifen in Duchenne muscular dystrophy (TAMDMD): Study protocol for a multicenter, randomized, placebo controlled, double-blind phase 3 trial","volume":"20","author":"Nagy","year":"2019","journal-title":"Trials"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.nmd.2008.06.386","article-title":"Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone","volume":"18","author":"Turgeman","year":"2008","journal-title":"Neuromuscul. Disord."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"H1550","DOI":"10.1152\/ajpheart.01253.2007","article-title":"Functional resolution of fibrosis in mdx mouse dystrophic heart and skeletal muscle by halofuginone","volume":"294","author":"Huebner","year":"2008","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"ref_95","first-page":"791","article-title":"Hydroxy group requirement for halofuginone-dependent inhibition of muscle fibrosis and improvement of histopathology in the mdx mouse model for Duchenne muscular dystrophy","volume":"34","author":"Wellner","year":"2019","journal-title":"Histol. Histopathol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1002\/ana.21338","article-title":"A phase I\/IItrial of MYO-029 in adult subjects with muscular dystrophy","volume":"63","author":"Wagner","year":"2008","journal-title":"Ann. Neurol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/s13395-017-0141-y","article-title":"A mouse anti-myostatin antibody increases muscle mass and improves muscle strength and contractility in the mdx mouse model of Duchenne muscular dystrophy and its humanized equivalent, domagrozumab (PF-06252616), increases muscle volume in cynomolgus monkeys","volume":"7","author":"Andre","year":"2017","journal-title":"Skelet. Muscle"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1038\/nature01154","article-title":"Functional improvement of dystrophic muscle by myostatin blockade","volume":"420","author":"Bogdanovich","year":"2002","journal-title":"Nature"},{"key":"ref_99","doi-asserted-by":"crossref","unstructured":"Wang, R., Jiao, H., Zhao, J., Wang, X., and Lin, H. (2016). Glucocorticoids enhance muscle proteolysis through a myostatin-dependent pathway at the early stage. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0156225"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"315","DOI":"10.2108\/zsj.26.315","article-title":"Expression of myostatin and follistatin in Mdx mice, an animal model for muscular dystrophy","volume":"26","author":"Abe","year":"2009","journal-title":"Zool. Sci."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/s13395-018-0180-z","article-title":"Myostatin and activin blockade by engineered follistatin results in hypertrophy and improves dystrophic pathology in mdx mouse more than myostatin blockade alone","volume":"8","author":"Iskenderian","year":"2018","journal-title":"Skelet. Muscle"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"4929","DOI":"10.1093\/hmg\/ddt342","article-title":"Micro-dystrophin and follistatin co-delivery restores muscle function in aged DMD model","volume":"22","author":"Janssen","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"4318","DOI":"10.1073\/pnas.0709144105","article-title":"Long-term enhancement of skeletal muscle mass and strength by single gene administration of myostatin inhibitors","volume":"105","author":"Haidet","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"e156","DOI":"10.1038\/mtna.2014.7","article-title":"Targeting TGF-\u03b2 signaling by antisense oligonucleotide-mediated knockdown of TGF-\u03b2 Type I receptor","volume":"3","author":"Kemaladewi","year":"2014","journal-title":"Mol. Ther.\u2014Nucleic Acids"},{"key":"ref_105","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":"Sun","year":"2008","journal-title":"J. Neurol. Sci."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"4938","DOI":"10.1093\/hmg\/ddt352","article-title":"Reducing CTGF\/CCN2 slows down mdx muscle dystrophy and improves cell therapy","volume":"22","author":"Morales","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_107","first-page":"867273","article-title":"Evidence of insulin resistance and other metabolic alterations in boys with Duchenne or Becker muscular dystrophy","volume":"2015","author":"Sanchez","year":"2015","journal-title":"Int. J. Endocrinol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1097\/JES.0b013e318201f236","article-title":"Heat shock proteins are important mediators of skeletal muscle insulin sensitivity","volume":"39","author":"Geiger","year":"2011","journal-title":"Exerc. Sport Sci. Rev."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1038\/nature10980","article-title":"Hsp72 preserves muscle function ans slows progression of severe muscular dystrophy","volume":"484","author":"Gehrig","year":"2012","journal-title":"Nature"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.afos.2015.07.008","article-title":"The effect of glucocorticoids on bone and muscle","volume":"1","author":"Klein","year":"2015","journal-title":"Osteoporos. Sarcopenia"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/1743-7075-2-3","article-title":"The role of glucocorticoid action in the pathophysiology of the Metabolic Syndrome","volume":"2","author":"Wang","year":"2005","journal-title":"Nutr. Metab."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1007\/s11102-013-0483-3","article-title":"Managing hyperglycemia in patients with Cushing\u2019s disease treated with pasireotide: Medical expert recommendations","volume":"17","author":"Colao","year":"2014","journal-title":"Pituitary"},{"key":"ref_113","doi-asserted-by":"crossref","unstructured":"Bostock, E.L., Edwards, B.T., Jacques, M.F., Pogson, J.T.S., Reeves, N.D., Onambele-Pearson, G.L., and Morse, C.I. (2018). Impaired glucose tolerance in adults with Duchenne and Becker muscular dystrophy. Nutrients, 10.","DOI":"10.3390\/nu10121947"},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1016\/S0387-7604(12)80071-4","article-title":"Glucose, free fatty acid and ketone body metabolism in Duchenne muscular dystrophy","volume":"12","author":"Nishio","year":"1990","journal-title":"Brain Dev."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1007\/s11307-018-1174-2","article-title":"Glucose metabolism as a pre-clinical biomarker for the Golden Retriever Model of Duchenne muscular dystrophy","volume":"20","author":"Schneider","year":"2018","journal-title":"Mol. Imaging Biol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1111\/j.1399-5448.2009.00512.x","article-title":"Metformin therapy to reduce weight gain and visceral adiposity in children and adolescents with neurogenic or myogenic motor deficit","volume":"11","author":"Casteels","year":"2010","journal-title":"Pediatr. Diabetes"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.bcp.2018.04.022","article-title":"Effect of a long-term treatment with metformin in dystrophic mdx mice: A reconsideration of its potential clinical interest in Duchenne muscular dystrophy","volume":"154","author":"Mantuano","year":"2018","journal-title":"Biochem. Pharmacol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"2163","DOI":"10.1016\/j.celrep.2018.10.077","article-title":"AMPK activation regulates LTBP4-dependent TGF-b1 secretion by pro-inflammatory macrophages and controls fibrosis in Duchenne muscular dystrophy","volume":"25","author":"Juban","year":"2018","journal-title":"Cell Rep."},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Langone, F., Cannata, S., Fuoco, C., Barbato, D., Testa, S., Nardozza, A., Ciriolo, M., Castagnoli, L., Cargioli, C., and Cesareni, G. (2014). Metformin protects skeletal muscle from cardiotoxin induced degeneration. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0114018"},{"key":"ref_120","doi-asserted-by":"crossref","unstructured":"Hafner, P., Bonati, U., Erne, B., Schmid, M., Rubino, D., Pohlman, U., Peters, T., Rutz, E., Frank, S., and Neuhaus, C. (2016). Improved muscle function in Duchenne muscular dystrophy through l-Arginine and metformin: An investigator-initiated, open-label, single-center, proof-of-concept-study. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0147634"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"e1914171","DOI":"10.1001\/jamanetworkopen.2019.14171","article-title":"Effect of combination l-citrulline and metformin treatment on motor function in patients with Duchenne muscular dystrophy: A randomized clinical trial","volume":"2","author":"Hafner","year":"2019","journal-title":"JAMA Netw. Open"},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/s13395-015-0051-9","article-title":"Involvement of adiponectin in the pathogenesis of dystrophinopathy","volume":"5","author":"Lecompte","year":"2015","journal-title":"Skelet. Muscle"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"6458","DOI":"10.1093\/hmg\/ddu366","article-title":"Discovery of serum protein biomarkers in the mdx mouse model and cross-species comparison to Duchenne muscular dystrophy patients","volume":"23","author":"Hathout","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"12167","DOI":"10.1038\/s41598-019-48548-9","article-title":"Disease-specific and glucocorticoid-responsive serum biomarkers for Duchenne muscular dystrophy","volume":"9","author":"Hathout","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1016\/j.ajpath.2017.02.018","article-title":"Potential therapeutic action of adiponectin in Duchenne muscular dystrophy","volume":"187","author":"Boursereau","year":"2017","journal-title":"Am. J. Pathol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1002\/jcsm.12531","article-title":"AdipoRon, a new therapeutic prospect for Duchenne muscular dystrophy","volume":"11","author":"Selvais","year":"2020","journal-title":"J. Cachexia Sarcopenia Muscle"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"3246","DOI":"10.1016\/j.ajpath.2016.08.008","article-title":"BGP-15 improves aspects of the dystrophic pathology in mdx and dko mice with differing efficacies in heart and skeletal muscle","volume":"186","author":"Kennedy","year":"2016","journal-title":"Am. J. Pathol."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.phrs.2018.09.024","article-title":"Idebenone is a cytoprotective insulin sensitizer whose mechanism is Shc inhibition","volume":"137","author":"Tomilov","year":"2018","journal-title":"Pharmacol. Res."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1093\/eurheartj\/ehn406","article-title":"Long-term blinded placebo-controlled study of SNT-MC17\/idebenone in the dystrophin deficient mdx mouse: Cardiac protection and improved exercise performance","volume":"30","author":"Buyse","year":"2009","journal-title":"Eur. Heart J."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"189","DOI":"10.3233\/JND-170245","article-title":"for the DELOS study Group. Efficacy of idebenone to preserve respiratory function above clinically meaningful thresholds for forced vital capacity (fvc) in patients with duchenne muscular dystrophy","volume":"4","author":"Mayer","year":"2017","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1002\/ppul.23547","article-title":"Treatment effect of idebenone on inspiratory function in patients with duchenne muscular dystrophy","volume":"52","author":"Buyse","year":"2017","journal-title":"Pediatr. Pulmonol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.nmd.2016.05.008","article-title":"Idebenone reduces respiratory complications in patients with duchenne muscular dystrophy","volume":"26","author":"McDonald","year":"2016","journal-title":"Neuromuscul. Disord."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"4609","DOI":"10.1074\/jbc.M407498200","article-title":"FK506-binding proteins 51 and 52 differentially regulate dynein interaction and nuclear translocation of the glucocorticoid receptor in mammalian cells","volume":"280","author":"Wochnik","year":"2005","journal-title":"J. Biol.Chem."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1093\/emboj\/cdg108","article-title":"The hsp90-binding peptidylprolyl isomerase FKBP52 potentiates glucocorticoid signaling in vivo","volume":"22","author":"Riggs","year":"2003","journal-title":"EMBO"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"4597","DOI":"10.1074\/jbc.C100531200","article-title":"A new first step in activation of steroid receptors: Hormone-induced switching of FKBP51 and FKBP52 immunophilins","volume":"15","author":"Davies","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"19183","DOI":"10.1073\/pnas.0805514105","article-title":"HDAC2 blockade by nitric oxide and histone deacetylase inhibitors reveals a common target in Duchenne muscular dystrophy treatment","volume":"105","author":"Colussi","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"547","DOI":"10.2217\/epi.14.36","article-title":"Histone deacetylase inhibitors: A potential epigenetic treatment for Duchenne muscular dystrophy","volume":"6","author":"Consalvi","year":"2014","journal-title":"Epigenomics"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1002\/emmm.201202096","article-title":"Fibroadipogenic progenitors mediate the ability of HDAC inhibitors to promote regeneration in dystrophic muscles of young, but not old mdx mice","volume":"5","author":"Mozzetta","year":"2013","journal-title":"EMBO Mol. Med."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1038\/nm1479","article-title":"Functional and morphological recovery of dystrophic muscles in mice treated with deacetylase inhibitors","volume":"12","author":"Minetti","year":"2006","journal-title":"Nat. Med."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"79","DOI":"10.2119\/molmed.2013.00011","article-title":"Preclinical studies in the mdx mouse model of Duchenne muscular dystrophy with the histone deacetylase inhibitor Givinostat","volume":"19","author":"Consalvi","year":"2013","journal-title":"Mol. Med."},{"key":"ref_141","first-page":"125","article-title":"HDAC inhibitors for muscular dystrophies: Progress and prospects","volume":"4","author":"Consalvi","year":"2015","journal-title":"Expert Opin. Orphan Drugs"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/j.nmd.2016.07.002","article-title":"Histological effects of givinostat in boys with Duchenne muscular dystrophy","volume":"26","author":"Bettica","year":"2016","journal-title":"Neuromuscul. Disord."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"2751","DOI":"10.3390\/ph3092751","article-title":"Clinical toxicities of histone deacetylase inhibitors","volume":"3","author":"Subramanian","year":"2010","journal-title":"Pharmaceuticals"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"5487","DOI":"10.1113\/jphysiol.2012.240390","article-title":"Peroxisome proliferator-activated receptor \u03b3 coactivator 1-\u03b1 gene transfer restores mitochondrial biomass and improves mitochondrial calcium handling in post-necrotic mdx mouse skeletal muscle","volume":"590","author":"Godin","year":"2012","journal-title":"J. Physiol."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"4640","DOI":"10.1093\/hmg\/ddp431","article-title":"Pharmacological activation of PPARbeta\/delta stimulates utrophin A expression in skeletal muscle fibers and restores sarcolemmal integrity in mature mdx mice","volume":"18","author":"Miura","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"e1312","DOI":"10.1212\/WNL.0000000000008168","article-title":"Vamorolone trial in Duchenne muscular dystrophy shows dose-related improvement of muscle function","volume":"93","author":"Hoffman","year":"2019","journal-title":"Neurology"},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"S5","DOI":"10.1542\/peds.2018-0333C","article-title":"Neurology care, diagnostics, and emerging therapies of the patient with Duchenne muscular dystrophy","volume":"142","author":"Leigh","year":"2018","journal-title":"Pediatrics"},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"29","DOI":"10.3233\/JND-150113","article-title":"Current translational research and murine models for Duchenne muscular dystrophy","volume":"3","author":"Rodrigues","year":"2016","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1124\/dmd.30.9.1029","article-title":"Glucocorticoid-mediated induction of CYP3A4 is decreased by disruption of a protein: DNA interaction distinct from the pregnane X receptor response element","volume":"30","author":"Bombail","year":"2002","journal-title":"Drug Metab. Dispos."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"a022301","DOI":"10.1101\/cshperspect.a022301","article-title":"Targeting TGF-\u03b2 signaling for therapeutic gain","volume":"9","author":"Akhurst","year":"2017","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1186\/s42269-019-0227-2","article-title":"Toll-like receptors activation, signaling and targeting: An overview","volume":"43","author":"Sibaii","year":"2019","journal-title":"Bull. Natl. Res. Cent."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1038\/sj.cr.7290105","article-title":"MAPK signal pathways in the regulation of cell proliferation in mammalian cells","volume":"12","author":"Zhang","year":"2002","journal-title":"Cell Res."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1136\/annrheumdis-2019-216487","article-title":"Efficacy and safety of NI-0101, an anti-toll-like receptor 4 monoclonal antibody, in patients with rheumatoid arthritis after inadequate response to methotrexate: A phase II study","volume":"79","author":"Monnet","year":"2020","journal-title":"Ann. Rheum. Dis."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/s13395-019-0207-0","article-title":"miR-146a deficiency does not aggravate muscular dystrophy in mdx mice","volume":"9","author":"Chwalenia","year":"2019","journal-title":"Skelet. Muscle"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF02990939","article-title":"Matrix metalloproteinase inhibitors in the treatment of cancer","volume":"14","author":"Brown","year":"1997","journal-title":"Med. Oncol."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1002\/humu.20918","article-title":"Theorectic applicability of antisense-mediated exon skipping for Duchenne muscular dystrophy mutations","volume":"30","author":"Fokkema","year":"2009","journal-title":"Hum. Mutat."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1146\/annurev-genom-091212-153527","article-title":"Therapeutics based on stop codon readthrough","volume":"15","author":"Keeling","year":"2017","journal-title":"Annu. Rev. Genom. Hum. Genet."},{"key":"ref_158","first-page":"301","article-title":"Therapeutic applications of CRISPR\/Cas for Duchenne muscular dystrophy","volume":"17","author":"Wong","year":"2017","journal-title":"Curr. Gene Ther."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1038\/nrd3625","article-title":"RNA therapeutics: Beyond RNA interference and antisense oligonucletides","volume":"11","author":"Kole","year":"2012","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"eaay6812","DOI":"10.1126\/sciadv.aay6812","article-title":"Enhanced CRISPR\/Cas9 correction of Duchenne muscular dystrophy in mice by a self-complementary AAV delivery system","volume":"6","author":"Zhang","year":"2020","journal-title":"Sci. Adv."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1177\/1756285615586123","article-title":"Duchenne muscular dystrophy: Current cell therapies","volume":"8","author":"Sienkiewicz","year":"2015","journal-title":"Ther. Adv. Neurol. 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