Article
PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress.
Disease models & mechanisms - 1 Jan 2011
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
Abstract excerpt
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...
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