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

• Main Authors: C. Catharina Müller (Author), Tam H. Nguyen (Author), Barbara Ahlemeyer (Author), Mallika Meshram (Author), Nishreen Santrampurwala (Author), Siyu Cao (Author), Peter Sharp (Author), Pamela B. Fietz (Author), Eveline Baumgart-Vogt (Author), Denis I. Crane (Author)
• Format: Book
• Published: The Company of Biologists, 2011-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

SUMMARY 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...