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Osteoblast malfunction caused by cell stress response to procollagen misfolding in α2(I)-G610C mouse model of osteogenesis imperfecta

Glycine (Gly) substitutions in collagen Gly-X-Y repeats disrupt folding of type I procollagen triple helix and cause severe bone fragility and malformations (osteogenesis imperfecta, aka OI). However, these mutations do not elicit the expected Endoplasmic Reticulum (ER) stress response, in contrast...

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Detalles Bibliográficos
Publicado en:J Bone Miner Res
Autores principales: Mirigian, Lynn S., Makareeva, Elena, Mertz, Edward L., Omari, Shakib, Roberts-Pilgrim, Anna M., Oestreich, Arin K., Phillips, Charlotte L., Leikin, Sergey
Formato: Artigo
Lenguaje:Inglês
Publicado: 2016
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC5061462/
https://ncbi.nlm.nih.gov/pubmed/26925839
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jbmr.2824
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