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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
5681
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pubmed:dateCreated |
2004-7-12
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pubmed:abstractText |
Numerous degenerative disorders are associated with elevated levels of prooxidants and declines in mitochondrial aconitase activity. Deficiency in the mitochondrial iron-binding protein frataxin results in diminished activity of various mitochondrial iron-sulfur proteins including aconitase. We found that aconitase can undergo reversible citrate-dependent modulation in activity in response to pro-oxidants. Frataxin interacted with aconitase in a citrate-dependent fashion, reduced the level of oxidant-induced inactivation, and converted inactive [3Fe-4S]1+ enzyme to the active [4Fe-4S]2+ form of the protein. Thus, frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
1095-9203
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pubmed:author |
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pubmed:issnType |
Electronic
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pubmed:day |
9
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pubmed:volume |
305
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
242-5
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:15247478-Aconitate Hydratase,
pubmed-meshheading:15247478-Animals,
pubmed-meshheading:15247478-Citric Acid,
pubmed-meshheading:15247478-Dithiothreitol,
pubmed-meshheading:15247478-Electron Spin Resonance Spectroscopy,
pubmed-meshheading:15247478-Enzyme Activation,
pubmed-meshheading:15247478-Ferrous Compounds,
pubmed-meshheading:15247478-Hydrogen Peroxide,
pubmed-meshheading:15247478-Iron,
pubmed-meshheading:15247478-Iron-Binding Proteins,
pubmed-meshheading:15247478-Male,
pubmed-meshheading:15247478-Mitochondria,
pubmed-meshheading:15247478-Mitochondria, Heart,
pubmed-meshheading:15247478-Molecular Chaperones,
pubmed-meshheading:15247478-Oxidation-Reduction,
pubmed-meshheading:15247478-Oxidative Stress,
pubmed-meshheading:15247478-Oxygen Consumption,
pubmed-meshheading:15247478-Rats,
pubmed-meshheading:15247478-Rats, Sprague-Dawley,
pubmed-meshheading:15247478-Saccharomyces cerevisiae,
pubmed-meshheading:15247478-Saccharomyces cerevisiae Proteins
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pubmed:year |
2004
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pubmed:articleTitle |
Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity.
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pubmed:affiliation |
Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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