Article
Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum.
The FEBS journal - 1 Dec 2015
Moriconi Claudia, Ordoñez Adriana, Lupo Giuseppe, Gooptu Bibek, Irving James A, Noto Rosina, Martorana Vincenzo, Manno Mauro, Timpano Valentina, Guadagno Noemi A, Dalton Lucy, Marciniak Stefan J, Lomas David A, Miranda Elena
Abstract excerpt
The neuronal serpin neuroserpin undergoes polymerisation as a consequence of point mutations that alter its conformational stability, leading to a neurodegenerative dementia called familial encephalopathy with neuroserpin inclusion bodies (FENIB). Neuroserpin is a glycoprotein with predicted glycosylation sites at asparagines 157, 321 and 401. We used site-directed mutagenesis, transient transfection, western...
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