Papers And Originals

Instability of Polymeric Skin Collagen in Osteogenesis Imperfecta

Br Med J 1974; 1 doi: (Published 09 March 1974) Cite this as: Br Med J 1974;1:421
  1. M. J. O. Francis,
  2. Roger Smith,
  3. Robert J. Bauze


    The structural polymeric collagen of the skin of 19 patients with osteogenesis imperfecta has been examined. In those with severe bone disease, who often have white sclerae, this collagen fraction is less resistant to depolymerization than that of age-matched controls, though the total amount is normal. In patients with less severe bone disease, whose sclerae are usually blue, the polymeric collagen may have normal stability but the total amount is reduced. These results suggest defective cross-linking of collagen in severe osteogenesis imperfecta.