Collagen defects in lethal perinatal osteogenesis imperfecta.

  title={Collagen defects in lethal perinatal osteogenesis imperfecta.},
  author={John F Bateman and D. P. S. Chan and Tom Mascara and John G. Rogers and William G. Cole},
  journal={The Biochemical journal},
  volume={240 3},
Quantitative and qualitative abnormalities of collagen were observed in tissues and fibroblast cultures from 17 consecutive cases of lethal perinatal osteogenesis imperfecta (OI). The content of type I collagen was reduced in OI dermis and bone and the content of type III collagen was also reduced in the dermis. Normal bone contained 99.3% type I and 0.7% type V collagen whereas OI bone contained a lower proportion of type I, a greater proportion of type V and a significant amount of type III… CONTINUE READING