Glycosylation tunes neuroserpin physiological and pathological properties
Neuroserpin (NS) is a member of the serine protease inhibitors superfamily. Specific point
mutations are responsible for its accumulation in the endoplasmic reticulum of neurons that leads to
a pathological condition named familial encephalopathy with neuroserpin inclusion bodies (FENIB).
Wild-type NS presents two N-glycosylation chains and does not form polymers in vivo, while
non-glycosylated NS causes aberrant polymer accumulation in cell models. To date, all in vitro