Deposition of mutant type I collagen in the extracellular matrix of cultured dermal fibroblasts in osteogenesis imperfecta.

doi:10.3109/03008209309061965

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Article

Deposition of mutant type I collagen in the extracellular matrix of cultured dermal fibroblasts in osteogenesis imperfecta.

Connective tissue research - 1 Jan 1993

Cetta G, Rossi A, Tenni R, Valli M, Forlino A, Zanaboni G, Dyne K, Burgio G R

Abstract excerpt

To study how mutant type I collagen interferes with matrix deposition we investigated the extracellular matrix produced by cultured skin fibroblasts in thirteen patients affected by different forms of Osteogenesis Imperfecta. Two different approaches were used: a) the pericellular matrix produced...

Topics

Cells, Cultured
Collagen
Electrophoresis, Polyacrylamide Gel
Extracellular Matrix
Fibroblasts
Humans
Mutation
Osteogenesis Imperfecta
Peptide Mapping
Phenotype
Skin

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