Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta.
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.
D002478:Cells, Cultured; D002852:Chromatography, Ion Exchange; D003094:Collagen / Q000032:analysis / Q000096:biosynthesis*; D005260:Female; D005347:Fibroblasts; D006801:Humans; D007223:Infant; D007700:Kinetics; D010013:Osteogenesis Imperfecta / Q000378:metabolism*; D012867:Skin / Q000378:metabolism*
D003094:Collagen