TNF and IL6/Jak2 signaling pathways are the main contributors of the glia-derived neuroinflammation present in Lafora disease, a fatal form of progressive myoclonus epilepsy

TNF and IL6/Jak2 signaling pathways are the main contributors of the glia-derived neuroinflammation present in Lafora disease, a fatal form of progressive myoclonus epilepsy

Lafora disease (LD; OMIM#254780) is a rare form of progressive myoclonus epilepsy (prevalence <1:1,000,000) characterized by the accumulation of insoluble deposits of aberrant glycogen (polyglucosans), named Lafora bodies, in the brain but also in peripheral tissues. LD is the most severe form of...

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Bibliographic Details
Main Authors: Teresa Rubio, Rosa Viana, Mireia Moreno-Estellés, Ángela Campos-Rodríguez, Pascual Sanz
Format: Article
Language:English
Published: Elsevier 2023-01-01
Series:Neurobiology of Disease
Subjects:
Lafora disease
Astrocytes
Microglia
Neuroinflammation
TNF
IL6
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996122003564

Internet

http://www.sciencedirect.com/science/article/pii/S0969996122003564

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