Cp/Heph mutant mice have iron-induced neurodegeneration diminished by deferiprone
Liangliang Zhao, Majda Hadziahmetovic, Chenguang Wang, Xueying Xu, Ying Song, H.A. Jinnah, Jolanta Wodzinska, Jared Iacovelli, Natalie Wolkow, Predrag Krajacic, Alyssa Cwanger Weissberger, John Connelly, Michael Spino, Michael K. Lee, James Connor, Benoit Giasson, Z. Leah Harris and Joshua L. Dunaief
Article first published online: 29 SEP 2015 | DOI: 10.1111/jnc.13292
Above: Iron (Fe) normally moves from capillaries to glia to neurons. It is exported from the glia by ferroportin (Fpn) with ferroxidases ceruloplasmin (Cp) and/or Hephaestin (Heph). Below: In mice with mutation of Cp and Heph, iron accumulates in glia, while neurons have low iron levels. Both neurons and glia degenerate and mice become ataxic unless given an iron chelator.