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Altered processing of alpha (I) collagen in a case of lethal osteogenesis imperfecta
The Italian Journal of Biochemistry
|September 1, 1986
Abstract:
Cultured skin fibroblasts from a newborn with the lethal perinatal form of Osteogenesis imperfecta synthesized an over-hydroxylated form of pro alpha 1 (I) chain. The analysis of the CNBr peptides showed that over-hydroxylation occurred all along the molecule.
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