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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...
Autores principales: | Bottelbergs, Astrid, Verheijden, Simon, Van Veldhoven, Paul P, Just, Wilhelm, Devos, Rita, Baes, Myriam |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2012
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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 |
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