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Peroxisome deficiency but not the defect in ether lipid synthesis causes activation of the innate immune system and axonal loss in the central nervous system

BACKGROUND: Mice with peroxisome deficiency in neural cells (Nestin-Pex5(−/−)) develop a neurodegenerative phenotype leading to motor and cognitive disabilities and early death. Major pathologies at the end stage of disease include severe demyelination, axonal degeneration and neuroinflammation. We...

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Detalles Bibliográficos
Autores principales: Bottelbergs, Astrid, Verheijden, Simon, Van Veldhoven, Paul P, Just, Wilhelm, Devos, Rita, Baes, Myriam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3419640/
https://www.ncbi.nlm.nih.gov/pubmed/22458306
http://dx.doi.org/10.1186/1742-2094-9-61