Abstract

Osteogenesis imperfecta (OI) is a rare disorder of connective tissue caused by abnormalities in the synthesis or processing of type I collagen. Type I collagen is the most abundant type of collagen and is expressed in almost all connective tissues. Given that type I collagen interacts with other collagens based in the extracellular matrix (ECM), we hypothesized changes in type I collagen in OI would result in perturbations in the homeostasis of other collagen types. We measured serum biomarkers of several non-type I collagens in patients with mild (type I) and moderate-to-severe (type III/IV) OI. Compared to controls, those with moderate-to severe OI had a higher mean level of the synthesis markers of collagen III (ProC3) (P = 0.02), and levels of collagen V (ProC5) (P = 0.07) were slightly, but not significantly, higher. Degradation markers of collage type IV (C4M2) (P = 0.04) and type VI (C6M) (P = 0.003) were also higher. In each case, a test for trend suggested levels were higher in moderate-to-severe OI, intermediate in mild OI, and lowest in controls (P = 0.06-0.002). These changes supports the hypothesis that mutations in type I collagen induce a widespread alteration in the ECM, and that the diverse clinical manifestations of OI reflect an extensive disruption in ECM biology.

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