Sil1-deficient Fibroblasts Generate an Aberrant Extracellular Matrix Leading to Tendon Disorganisation in Marinesco-Sjögren Syndrome

Laura Amodei,Anna Giulia Ruggieri, Francesca Potenza, Marianna Viele,Beatrice Dufrusine, Raffaella Franciotti,Laura Pietrangelo,Matteo Ardini,Liborio Stuppia,Luca Federici,Vincenzo De Laurenzi,Michele Sallese

Journal of Translational Medicine（2024）

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关键词

Endoplasmic reticulum,ER stress,Extracellular matrix,Unfolded protein response,Collagen,Cytoskeleton,Transcriptomic analysis,DQ-collagen,Electron micrograph,Wound-healing,Cell motility,Cell attachment,Woozy mouse,Skeletal muscle,Tendon

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