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Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases

Mutant matrilin-3 (V194D) forms non-native disulphide bonded aggregates in the rER of chondrocytes from cell and mouse models of multiple epiphyseal dysplasia (MED). Intracellular retention of mutant matrilin-3 causes endoplasmic reticulum (ER) stress and induces an unfolded protein response (UPR) i...

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Detalles Bibliográficos
Autores principales:	Hartley, Claire L., Edwards, Sarah, Mullan, Lorna, Bell, Peter A., Fresquet, Maryline, Boot-Handford, Raymond P., Briggs, Michael D.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2013
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3842181/ https://www.ncbi.nlm.nih.gov/pubmed/23956175 http://dx.doi.org/10.1093/hmg/ddt383

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