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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Main Authors: 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.
Format: Artigo
Sprog:Inglês
Udgivet: Oxford University Press 2013
Fag:
Articles
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3889810/
https://ncbi.nlm.nih.gov/pubmed/23814041
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddt310
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