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

• 主要な著者: C. Catharina Müller (著者), Tam H. Nguyen (著者), Barbara Ahlemeyer (著者), Mallika Meshram (著者), Nishreen Santrampurwala (著者), Siyu Cao (著者), Peter Sharp (著者), Pamela B. Fietz (著者), Eveline Baumgart-Vogt (著者), Denis I. Crane (著者)
• フォーマット: 図書
• 出版事項: The Company of Biologists, 2011-01-01T00:00:00Z.
• 主題: article
• オンライン･アクセス: 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...