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http://purl.uniprot.org/citations/30463013http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/30463013http://www.w3.org/2000/01/rdf-schema#comment"Chronic inflammation and fibrosis characterize Duchenne muscular dystrophy (DMD). We show that pro-inflammatory macrophages are associated with fibrosis in mouse and human DMD muscle. DMD-derived Ly6Cpos macrophages exhibit a profibrotic activity by sustaining fibroblast production of collagen I. This is mediated by the high production of latent-TGF-β1 due to the higher expression of LTBP4, for which polymorphisms are associated with the progression of fibrosis in DMD patients. Skewing macrophage phenotype via AMPK activation decreases ltbp4 expression by Ly6Cpos macrophages, blunts the production of latent-TGF-β1, and eventually reduces fibrosis and improves DMD muscle force. Moreover, fibro-adipogenic progenitors are the main providers of TGF-β-activating enzymes in mouse and human DMD, leading to collagen production by fibroblasts. In vivo pharmacological inhibition of TGF-β-activating enzymes improves the dystrophic phenotype. Thus, an AMPK-LTBP4 axis in inflammatory macrophages controls the production of TGF-β1, which is further activated by and acts on fibroblastic cells, leading to fibrosis in DMD."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.org/dc/terms/identifier"doi:10.1016/j.celrep.2018.10.077"xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Desguerre I."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Arnold L."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Cuvellier S."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Mounier R."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Juban G."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Boisson C."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Magnan M."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Petrof B.J."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Chazaud B."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Ben Larbi S."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Gondin J."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Theret M."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Yacoub-Youssef H."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Saclier M."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/author"Kernou A."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/date"2018"xsd:gYear
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/name"Cell Rep"xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/pages"2163-2176.e6"xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/title"AMPK Activation Regulates LTBP4-Dependent TGF-beta1 Secretion by Pro-inflammatory Macrophages and Controls Fibrosis in Duchenne Muscular Dystrophy."xsd:string
http://purl.uniprot.org/citations/30463013http://purl.uniprot.org/core/volume"25"xsd:string
http://purl.uniprot.org/citations/30463013http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/30463013
http://purl.uniprot.org/citations/30463013http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/30463013