OI is characterized by disorders in collagen I metabolism. Biochemical aspects of OI have been studied mostly with skin fibroblasts although bone is the most affected tissue in OI. We studied collagen metabolism of osteoblasts in culture (OBC) derived from 30 OI patients (types I-IV). All OI OBC produced low lewels of collagen. Qualitative abnormal collagen I as determined by PAGE was found among all OI types. In single cases procollagen processing was slow. Many OI OBC showed a decreased pericellular accumulation of collagen I (coll.I).Conclusion: Decreased synthesis and pericellular accumulation of collagen was found associated with, but also irrespective of, obvious structural defects in collagen I and may play a crucial role in the phenotype of OI osteoblasts.
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