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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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| Publicado no: | J Bone Miner Res |
|---|---|
| Main Authors: | , , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
2016
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| Assuntos: | |
| Acesso em linha: | 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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