OBJECTIVES: Duchenne muscular dystrophy (DMD) is a degenerative muscle wasting disease caused by mutations in the dystrophin gene. Dystrophic muscle is characterized by chronic inflammation, and inflammatory mediators could be promising targets for innovative therapeutic interventions. We analyzed muscle biopsy samples of DMD-affected children to characterize interleukin (IL)-17 and Forkhead box P3 (Foxp3) expression levels and to identify possible correlations with clinical status. METHODS: Expression levels of IL-17, Foxp3, tumor necrosis factor-α (TNF-α), monocyte chemoattractant protein-1 (MCP-1), IL-6, and transforming growth factor-β (TGF-β) were analyzed by real-time PCR in muscle biopsy samples from patients with DMD (n = 27) and juvenile dermatomyositis (JDM) (n = 8). Motor outcome of patients with DMD was evaluated by North Star Ambulatory Assessment score. RESULTS: In DMD, we found higher levels of IL-17 and lower levels of Foxp3 mRNA compared with those for a typical inflammatory myopathy, JDM. Moreover, the IL-17/Foxp3 ratio was higher in DMD than in JDM biopsy samples. IL-17 mRNA levels appeared to be related to the expression levels of other proinflammatory cytokines (TNF-α and MCP-1) and significantly associated with clinical outcome of patients. CONCLUSIONS: The association of IL-17 expression with levels of other inflammatory cytokines and with the clinical course of DMD suggests a possible pathogenic role of IL-17.
OBJECTIVES: Duchenne muscular dystrophy (DMD) is a degenerative muscle wasting disease caused by mutations in the dystrophin gene. Dystrophic muscle is characterized by chronic inflammation, and inflammatory mediators could be promising targets for innovative therapeutic interventions. We analyzed muscle biopsy samples of DMD-affected children to characterize interleukin (IL)-17 and Forkhead box P3 (Foxp3) expression levels and to identify possible correlations with clinical status. METHODS: Expression levels of IL-17, Foxp3, tumor necrosis factor-α (TNF-α), monocyte chemoattractant protein-1 (MCP-1), IL-6, and transforming growth factor-β (TGF-β) were analyzed by real-time PCR in muscle biopsy samples from patients with DMD (n = 27) and juvenile dermatomyositis (JDM) (n = 8). Motor outcome of patients with DMD was evaluated by North Star Ambulatory Assessment score. RESULTS: In DMD, we found higher levels of IL-17 and lower levels of Foxp3 mRNA compared with those for a typical inflammatory myopathy, JDM. Moreover, the IL-17/Foxp3 ratio was higher in DMD than in JDM biopsy samples. IL-17 mRNA levels appeared to be related to the expression levels of other proinflammatory cytokines (TNF-α and MCP-1) and significantly associated with clinical outcome of patients. CONCLUSIONS: The association of IL-17 expression with levels of other inflammatory cytokines and with the clinical course of DMD suggests a possible pathogenic role of IL-17.
Authors: E Pegoraro; E P Hoffman; L Piva; B F Gavassini; S Cagnin; M Ermani; L Bello; G Soraru; B Pacchioni; M D Bonifati; G Lanfranchi; C Angelini; A Kesari; I Lee; H Gordish-Dressman; J M Devaney; C M McDonald Journal: Neurology Date: 2010-12-22 Impact factor: 9.910
Authors: Erik Lubberts; Marije I Koenders; Birgitte Oppers-Walgreen; Liduine van den Bersselaar; Christina J J Coenen-de Roo; Leo A B Joosten; Wim B van den Berg Journal: Arthritis Rheum Date: 2004-02
Authors: John S Tzartos; Manuel A Friese; Matthew J Craner; Jackie Palace; Jia Newcombe; Margaret M Esiri; Lars Fugger Journal: Am J Pathol Date: 2007-12-21 Impact factor: 4.307
Authors: Daniel P Reay; Sheldon I Bastacky; Kathryn E Wack; Donna B Stolz; Paul D Robbins; Paula R Clemens Journal: Mol Med Date: 2015-05-22 Impact factor: 6.354
Authors: David P Burns; Leonie Canavan; Jane Rowland; Robin O'Flaherty; Molly Brannock; Sarah E Drummond; Dervla O'Malley; Deirdre Edge; Ken D O'Halloran Journal: J Physiol Date: 2018-10-03 Impact factor: 5.182
Authors: Adonis Sfera; Carolina Osorio; Carlos M Zapata Martín Del Campo; Shaniah Pereida; Steve Maurer; Jose Campo Maldonado; Zisis Kozlakidis Journal: Front Cell Neurosci Date: 2021-06-25 Impact factor: 5.505