PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress

Delayed cerebellar development is a hallmark of Zellweger syndrome (ZS), a severe neonatal neurodegenerative disorder. ZS is caused by mutations in PEX genes, such as PEX13, which encodes a protein required for import of proteins into the peroxisome. The molecular basis of ZS pathogenesis is not kno...

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Detalles Bibliográficos
Autores principales: Müller, C. Catharina, Nguyen, Tam H., Ahlemeyer, Barbara, Meshram, Mallika, Santrampurwala, Nishreen, Cao, Siyu, Sharp, Peter, Fietz, Pamela B., Baumgart-Vogt, Eveline, Crane, Denis I.
Formato: Texto
Lenguaje:English
Publicado: The Company of Biologists Limited 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3014351/
https://www.ncbi.nlm.nih.gov/pubmed/20959636
http://dx.doi.org/10.1242/dmm.004622

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