Article
Lectin OS-9 delivers mutant neuroserpin to endoplasmic reticulum associated degradation in familial encephalopathy with neuroserpin inclusion bodies.
Neurobiology of aging - 1 Oct 2014
Schipanski Angela, Oberhauser Felix, Neumann Melanie, Lange Sascha, Szalay Beata, Krasemann Susanne, van Leeuwen Fred W, Galliciotti Giovanna, Glatzel Markus
Abstract excerpt
A feature of neurodegenerative diseases is the intraneuronal accumulation of misfolded proteins. In familial encephalopathy with neuroserpin inclusion bodies (FENIB), mutations in neuroserpin lead to accumulation of neuroserpin polymers within the endoplasmic reticulum (ER) of neurons. Cell culture based studies have shown that ER-associated degradation (ERAD) is involved in clearance of mutant neuroserpin. Here,...
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