Mössbauer studies of frataxin role in iron-sulfur cluster assembly and dysfunction-related disease
| Autor: | Ricardo Garcia-Serres, Jean-Marc Latour, Martin Clémancey, Jean-Louis Oddou, Annalisa Pastore, Emmanuel Lesuisse |
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| Rok vydání: | 2011 |
| Předmět: |
Iron-sulfur cluster assembly
chemistry.chemical_classification Nuclear and High Energy Physics biology Chemistry Mutant Iron–sulfur cluster Mitochondrion Gene mutation Condensed Matter Physics Atomic and Molecular Physics and Optics Ferritin chemistry.chemical_compound Biochemistry biology.protein Metalloprotein Frataxin Physical and Theoretical Chemistry |
| Zdroj: | Hyperfine Interactions. 206:13-18 |
| ISSN: | 1572-9540 0304-3843 |
| DOI: | 10.1007/s10751-011-0544-9 |
| Popis: | Friedreich ataxia is a disease that is associated with defects in the gene coding for a small protein frataxin. Several different roles have been proposed for the protein, including iron chaperoning and iron storage. Mossbauer spectroscopy was used to probe these hypotheses. Iron accumulation in mutant mitochondria unable to assemble iron sulfur clusters proved to be insensitive to overexpression of frataxin, ruling out its potential involvement as an iron storage protein similar to ferritin. Rather, it was found that frataxin negatively regulates iron sulfur cluster assembly. |
| Databáze: | OpenAIRE |
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