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Article

Osteoblast Malfunction Caused by Cell Stress Response to Procollagen Misfolding in α2(I)-G610C Mouse Model of Osteogenesis Imperfecta.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Aug 2016

Mirigian Lynn S, Makareeva Elena, Mertz Edward L, Omari Shakib, Roberts-Pilgrim Anna M, Oestreich Arin K, Phillips Charlotte L, Leikin Sergey

Abstract excerpt

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 [OI]). However, these mutations do not elicit the expected endoplasmic reticulum (ER) stress response, in contrast to other protein-folding diseases. Thus, it has remained unclear whether cell stress and osteoblast malfunction...

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Share to Collagen Type I
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 26925839
DOI: 10.1002/jbmr.2824
PMCID: PMC5061462

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