Collagen VI Deficiency Impairs Tendon Fibroblasts Mechanoresponse in Ullrich Congenital Muscular Dystrophy

Collagen VI Deficiency Impairs Tendon Fibroblasts Mechanoresponse in Ullrich Congenital Muscular Dystrophy

The pericellular matrix (PCM) is a specialized extracellular matrix that surrounds cells. Interactions with the PCM enable the cells to sense and respond to mechanical signals, triggering a proper adaptive response. Collagen VI is a component of muscle and tendon PCM. Mutations in collagen VI genes...

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Bibliographic Details
Main Authors: Vittoria Cenni, Patrizia Sabatelli, Alberto Di Martino, Luciano Merlini, Manuela Antoniel, Stefano Squarzoni, Simona Neri, Spartaco Santi, Samuele Metti, Paolo Bonaldo, Cesare Faldini
Format: Article
Language:English
Published: MDPI AG 2024-02-01
Series:Cells
Subjects:
collagen VI
Ullrich congenital muscular dystrophy
COL6-related myopathies
contractures
tendon fibroblasts
mechanosignaling
Online Access:https://www.mdpi.com/2073-4409/13/5/378

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https://www.mdpi.com/2073-4409/13/5/378

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