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. 1975 Feb;72(2):586-9.
doi: 10.1073/pnas.72.2.586.

Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta

R P Penttinen, J R Lichtenstein, G R Martin, V A McKusick

Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta

R P Penttinen et al. Proc Natl Acad Sci U S A. 1975 Feb.

Abstract

Cells obtained from normal human skin synthesize predominantly type I collogen in culture. Cells obtained from the skin of an infant with a severe form of osteogenesis imperfecta were found to synthesize as much type III as type I collagen. Decreased synthesis of type I collagen could explain the tissue fragility observed in this case.

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