Cytokines and Chemokines as Regulators of Skeletal Muscle Inflammation: Presenting the Case of Duchenne Muscular Dystrophy
Immunofluorescent detection of TNF-α and IFN-γ in Duchenne muscular dystrophy. (a)-(b) Muscle biopsy taken from an 8-year-old patient with Duchenne muscular dystrophy caused by duplication of dystrophin exon 2, resulting in severe muscle damage, few groups of revertant fibers, and strong utrophin staining. TNF-α (red in (a)) is detected in a small cluster of inflammatory cells and colocalizes with CD3 (green in (b)). The asterisk is an indicative that helps to identify an individual TNF-α+ T cell. (c)-(d) Muscle biopsy taken from a 2-year-old patient with Duchenne muscular dystrophy caused by c.5299-5302dupATTT in dystrophin exon 37. Myopathological evaluation of the biopsy described definite muscle damage, few groups of revertant fibers, and strong utrophin staining. IFN-γ (red in (c)) is strongly expressed on the blood vessel endothelium (arrow) and on perivascular CD3+ T cells (green in (d)). Highlighted are an IFN-γ+ CD3+ T cell attached to the luminal side of the blood vessel (asterisk) and an interstitial IFN-γ+ CD3+ T cell (circle).