These observations were supported by the quantitative analysis of the number of colloidal gold particles both in the PCM and in areas of tendon matrix displaced from the cells, which further demonstrated significant changes of COLVI, COLI and FBN distribution in the PCM of the UCMD tendon biopsy (Figure 2C). The gene discussed is FBN1; the disease is Congenital muscular dystrophy, Ullrich type.