Biochemical Analysis of a Hyperplastic Callus in Osteogenesis Imperfecta Type IV
Osteogenesis imperfecta (OI) is an inherited disease of connective tissue, which is characterized by a high fracture rate and skeletal deformations. In addition other tissues containing high amounts of type I collagen like the skin, sklerae and teeth can be affected. According to clinical features it has been divided into 4 different types (1). Various metabolic and structural abnormalities of type I collagen have been reported underlining the clinical heterogeneity. Fibroblasts of some patients produce overhydroxylated and overglycosylated type I collagen and in sporadic cases mutations of the α1 (I)- or α2 (I)-genes have been described (2).
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