Endoplasmic reticulum stress in amelogenesis imperfecta and phenotypic rescue using 4-phenylbutyrate

Inherited diseases caused by genetic mutations can arise due to loss of protein function. Alternatively, mutated proteins may mis-fold, impairing endoplasmic reticulum (ER) trafficking, causing ER stress and triggering the unfolded protein response (UPR). The UPR attempts to restore proteostasis but...

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Detalles Bibliográficos
Autores principales: Brookes, Steven J., Barron, Martin J., Boot-Handford, Ray, Kirkham, Jennifer, Dixon, Michael J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2014
Materias:
Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3976337/
https://www.ncbi.nlm.nih.gov/pubmed/24362885
http://dx.doi.org/10.1093/hmg/ddt642

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3976337/
https://www.ncbi.nlm.nih.gov/pubmed/24362885
http://dx.doi.org/10.1093/hmg/ddt642

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