The alteration of the FBN pattern in cultured tenocytes is consistent with our previous reports that COLVI deficiency affects the three-dimensional organization of FBN in fibroblast cultures of UCMD and Bethlem myopathy patients (Martoni et al., 2009), and in Col6A1−/− null mice (Sabatelli et al., 2001). The gene discussed is FBN1; the disease is Bethlem myopathy.