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Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB

Mutants of neuroserpin are retained as polymers within the endoplasmic reticulum (ER) of neurones to cause the autosomal dominant dementia familial encephalopathy with neuroserpin inclusion bodies or FENIB. The cellular consequences are unusual in that the ordered polymers activate the ER overload r...

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Detalles Bibliográficos
Autores principales:	Roussel, Benoit D., Newton, Timothy M., Malzer, Elke, Simecek, Nikol, Haq, Imran, Thomas, Sally E., Burr, Marian L., Lehner, Paul J., Crowther, Damian C., Marciniak, Stefan J., Lomas, David A.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2013
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3889810/ https://www.ncbi.nlm.nih.gov/pubmed/23814041 http://dx.doi.org/10.1093/hmg/ddt310

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