While future studies employing PC-specific inhibitors and tendon cultures from further patients with genetically defined mutations of COL6A1-A3 genes will provide a thorough dissection of the pathomolecular mechanisms involving cilia and Hh signaling defects in COL6-RM, our findings reveal, for the first time, an impairment of both PC hallmarks and Hh signaling in UCMD TFs during the exposure to mechanical stress. The gene discussed is COL6A1; the disease is Congenital muscular dystrophy, Ullrich type.