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A defect in the structure of type I procollagen in a patient who had osteogenesis imperfecta: excess mannose in the COOH-terminal propeptide.

Fibroblasts from normal human subjects and from a patient who had osteogenesis imperfecta were incubated with [3H]mannose, and types I and III procollagens were isolated from the culture medium. The type I procollagen from the patient's fibroblasts contained 2-3 time more [3H]mannose than the type I...

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Publicado en:Proc Natl Acad Sci U S A
Autores principales: Peltonen, L, Palotie, A, Prockop, D J
Formato: Artigo
Lenguaje:Inglês
Publicado: National Academy of Sciences 1980
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Acceso en línea:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC350238/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6934545/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.77.10.6179
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