Article
Loss of function mutations in the progranulin gene are related to pro-inflammatory cytokine dysregulation in frontotemporal lobar degeneration patients.
Journal of neuroinflammation - 6 Jun 2011
Bossù Paola, Salani Francesca, Alberici Antonella, Archetti Silvana, Bellelli Giuseppe, Galimberti Daniela, Scarpini Elio, Spalletta Gianfranco, Caltagirone Carlo, Padovani Alessandro, Borroni Barbara
Abstract excerpt
The progranulin gene (PGRN) encodes a pleiotropic molecule with anti-inflammatory actions and neuronal protective effects. Accordingly, PGRN-deficient mice have been demonstrated to develop enhanced inflammation and progressive neurodegeneration. Loss of function mutations of the PGRN gene have been also reported to cause frontotemporal lobar degeneration (FTLD), a neurodegenerative disease leading to dementia...
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