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- Title
The structure and function of frataxin.
- Authors
Bencze, Krisztina Z; Kondapalli, Kalyan C; Cook, Jeremy D; McMahon, Stephen; Millán-Pacheco, César; Pastor, Nina; Stemmler, Timothy L
- Abstract
Frataxin, a highly conserved protein found in prokaryotes and eukaryotes, is required for efficient regulation of cellular iron homeostasis. Humans with a frataxin deficiency have the cardio- and neurodegenerative disorder Friedreich's ataxia, commonly resulting from a GAA trinucleotide repeat expansion in the frataxin gene. While frataxin's specific function remains a point of controversy, the general consensus is that the protein assists in controlling cellular iron homeostasis by directly binding iron. This review focuses on the structural and biochemical aspects of iron binding by the frataxin orthologs and outlines molecular attributes that may help explain the protein's role in different cellular pathways.
- Publication
Critical reviews in biochemistry and molecular biology, 2006, Vol 41, Issue 5, p269
- ISSN
1040-9238
- Publication type
Journal Article
- DOI
10.1080/10409230600846058