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Article

G392E neuroserpin causing the dementia FENIB is secreted from cells but is not synaptotoxic.

Scientific reports - 22 Apr 2021

Ingwersen Thies, Linnenberg Christian, D'Acunto Emanuela, Temori Shabnam, Paolucci Irene, Wasilewski David, Mohammadi Behnam, Kirchmair Johannes, Glen Robert C, Miranda Elena, Glatzel Markus, Galliciotti Giovanna

Abstract excerpt

Familial encephalopathy with neuroserpin inclusion bodies (FENIB) is a progressive neurodegenerative disease caused by point mutations in the gene for neuroserpin, a serine protease inhibitor of the nervous system. Different mutations are known that are responsible for mutant neuroserpin polymerization and accumulation as inclusion bodies in many cortical and subcortical neurons, thereby leading to cell death,...

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Identifiers

PMID: 33888787
DOI: 10.1038/s41598-021-88090-1
PMCID: PMC8062559

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