Article
Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins.
The Journal of biological chemistry - 21 Aug 2009
Kroeger Heike, Miranda Elena, MacLeod Ian, Pérez Juan, Crowther Damian C, Marciniak Stefan J, Lomas David A
Abstract excerpt
The serpinopathies are a family of diseases characterized by the accumulation of ordered polymers of mutant protein within the endoplasmic reticulum. They are a diverse group including alpha(1)-antitrypsin deficiency and the inherited dementia familial encephalopathy with neuroserpin inclusion bodies or FENIB. We have used transient transfection of COS7 cells and mouse embryonic fibroblasts, PC12 cell lines that...
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