Article
The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.
Structure (London, England : 1993) - 1 Oct 2006
Karlberg Tobias, Schagerlöf Ulrika, Gakh Oleksandr, Park Sungjo, Ryde Ulf, Lindahl Martin, Leath Kirstin, Garman Elspeth, Isaya Grazia, Al-Karadaghi Salam
Abstract excerpt
Defects in the mitochondrial protein frataxin are responsible for Friedreich ataxia, a neurodegenerative and cardiac disease that affects 1:40,000 children. Here, we present the crystal structures of the iron-free and iron-loaded frataxin trimers, and a single-particle electron microscopy reconstruction of a 24 subunit oligomer. The structures reveal fundamental aspects of the frataxin mechanism. The trimer has a...
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