Article
Expression of mutant alpha (I)-procollagen in osteoblast and fibroblast cultures from a proband with osteogenesis imperfecta type IV.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Jul 1992
Chipman S D, Shapiro J R, McKinstry M B, Stover M L, Branson P, Rowe D W
Abstract excerpt
This study compares the synthesis of mutant type I collagen in cultured dermal fibroblasts and trabecular osteoblasts that were isolated from a patient with moderately severe osteogenesis imperfecta (type IV). Previous study of this patient's dermal fibroblasts revealed a 2000 dalton deletion located in cyanogen bromide peptide 4 of alpha 2(I)-collagen. The phenotype of the bone cell cultures was defined by a 3-4...
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