Tendon Extracellular Matrix Remodeling and Defective Cell Polarization in the Presence of Collagen VI Mutations - Antoniel, Manuela; Traina, Francesco; Merlini, Luciano; Andrenacci, Davide; Tigani, Domenico; Santi, Spartaco; Cenni, Vittoria; Sabatelli, Patrizia; Faldini, Cesare; Squarzoni, Stefano (Cells (Basel, Switzerland), 2020)
Keywords: Antigens - genetics; bethlem myopathy; Biopsy; Cell Polarity - genetics; cell polarization; cell-extracellular matrix interactions; Collagen Type VI - genetics; collagen vi; Contracture - diagnostic imaging; Contracture - genetics; Contracture - pathology; Extracellular Matrix - genetics; Extracellular Matrix - metabolism; extracellular matrix remodeling; Female; Fibroblasts - metabolism; Fibroblasts - pathology; Humans; Male; Matrix Metalloproteinase 2 - genetics; metalloproteinase 2; Muscle, Skeletal - pathology; Muscle, Skeletal - ultrastructure; Muscular Dystrophies - congenital; Muscular Dystrophies - diagnostic imaging; Muscular Dystrophies - genetics; Muscular Dystrophies - pathology; Mutation - genetics; ng2 proteoglycan; pericellular matrix; Proteoglycans - genetics; Sclerosis - diagnostic imaging; Sclerosis - genetics; Sclerosis - pathology; Tendons - diagnostic imaging; Tendons - pathology; Tendons - ultrastructure; ullrich congenital muscular dystrophy; collagen VI; NG2 proteoglycan; pericellular matrix; metalloproteinase 2; cell polarization; cell-extracellular matrix interactions; Ullrich congenital muscular dystrophy; Bethlem myopathy; extracellular matrix remodeling