Published in Biochemistry on May 27, 2003
The pathogenesis of Friedreich ataxia and the structure and function of frataxin. J Neurol (2009) 1.68
Monomeric yeast frataxin is an iron-binding protein. Biochemistry (2006) 1.67
The structure and function of frataxin. Crit Rev Biochem Mol Biol (2006) 1.59
Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities. Antioxid Redox Signal (2010) 1.27
Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia. Proc Natl Acad Sci U S A (2008) 1.22
Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis. J Biol Chem (2009) 1.13
PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia. PLoS One (2010) 1.11
Overexpression and characterization of an iron storage and DNA-binding Dps protein from Trichodesmium erythraeum. Appl Environ Microbiol (2006) 1.00
Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer. Biochemistry (2008) 0.98
Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level. J Biol Chem (2008) 0.91
Mapping brain metals to evaluate therapies for neurodegenerative disease. CNS Neurosci Ther (2010) 0.91
Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering. J Mol Biol (2011) 0.86
In silico pathway reconstruction: Iron-sulfur cluster biogenesis in Saccharomyces cerevisiae. BMC Syst Biol (2007) 0.83
The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization. J Biol Chem (2013) 0.80
Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: THE SUB-COMPLEX FORMED BY THE IRON DONOR, Yfh1 PROTEIN, AND THE SCAFFOLD, Isu1 PROTEIN. J Biol Chem (2016) 0.79
The Structure of the Complex between Yeast Frataxin and Ferrochelatase: CHARACTERIZATION AND PRE-STEADY STATE REACTION OF FERROUS IRON DELIVERY AND HEME SYNTHESIS. J Biol Chem (2016) 0.77
Magnetic properties and structural characterization of iron oxide nanoparticles formed by Streptococcus suis Dpr and four mutants. J Biol Inorg Chem (2011) 0.76
SAXS and stability studies of iron-induced oligomers of bacterial frataxin CyaY. PLoS One (2017) 0.75
Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers. Metallomics (2017) 0.75
The chemical form of mercury in fish. Science (2003) 3.55
CsoR is a novel Mycobacterium tuberculosis copper-sensing transcriptional regulator. Nat Chem Biol (2006) 3.30
Mitochondrial processing peptidases. Biochim Biophys Acta (2002) 2.53
Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science (2004) 2.29
Biochemistry: a cadmium enzyme from a marine diatom. Nature (2005) 1.88
Mechanisms of gold biomineralization in the bacterium Cupriavidus metallidurans. Proc Natl Acad Sci U S A (2009) 1.81
Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity. Hum Mol Genet (2005) 1.69
Spectroscopic studies of Pyrococcus furiosus superoxide reductase: implications for active-site structures and the catalytic mechanism. J Am Chem Soc (2002) 1.60
Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia. Hum Mol Genet (2002) 1.54
Molecular mimicry in mercury toxicology. Chem Res Toxicol (2006) 1.53
Increased glutathione biosynthesis plays a role in nickel tolerance in thlaspi nickel hyperaccumulators. Plant Cell (2004) 1.48
C-terminal domain of the membrane copper transporter Ctr1 from Saccharomyces cerevisiae binds four Cu(I) ions as a cuprous-thiolate polynuclear cluster: sub-femtomolar Cu(I) affinity of three proteins involved in copper trafficking. J Am Chem Soc (2004) 1.47
Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. J Biol Chem (2005) 1.44
Selenium biotransformations in an insect ecosystem: effects of insects on phytoremediation. Environ Sci Technol (2004) 1.40
Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J Biol Chem (2003) 1.39
High-throughput immunoassay for the biochemical diagnosis of Friedreich ataxia in dried blood spots and whole blood. Clin Chem (2013) 1.39
Human Sco1 and Sco2 function as copper-binding proteins. J Biol Chem (2005) 1.37
Assembly of human frataxin is a mechanism for detoxifying redox-active iron. Biochemistry (2005) 1.34
The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron. Structure (2006) 1.31
Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion. Proc Natl Acad Sci U S A (2005) 1.24
Reduction in frataxin causes progressive accumulation of mitochondrial damage. Hum Mol Genet (2003) 1.24
Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly. J Biol Chem (2010) 1.23
Physical evidence that yeast frataxin is an iron storage protein. Biochemistry (2002) 1.23
Copper transfer from the Cu(I) chaperone, CopZ, to the repressor, Zn(II)CopY: metal coordination environments and protein interactions. Biochemistry (2002) 1.20
A novel arsenate reductase from the arsenic hyperaccumulating fern Pteris vittata. Plant Physiol (2006) 1.19
The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast. Hum Mol Genet (2004) 1.19
Yeast Cox11, a protein essential for cytochrome c oxidase assembly, is a Cu(I)-binding protein. J Biol Chem (2002) 1.18
Chelatases: distort to select? Trends Biochem Sci (2006) 1.18
Prion protein expression level alters regional copper, iron and zinc content in the mouse brain. Metallomics (2011) 1.17
Polymorphisms in mitochondrial genes and prostate cancer risk. Cancer Epidemiol Biomarkers Prev (2008) 1.17
Cleavage site selection within a folded substrate by the ATP-dependent lon protease. J Biol Chem (2005) 1.16
Unnatural amino acid substitution as a probe of the allosteric coupling pathway in a mycobacterial Cu(I) sensor. J Am Chem Soc (2009) 1.14
Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis. J Biol Chem (2009) 1.13
Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers. Mol Genet Metab (2008) 1.12
X-ray absorption spectroscopy as a probe of microbial sulfur biochemistry: the nature of bacterial sulfur globules revisited. J Bacteriol (2008) 1.12
Mapping metals in Parkinson's and normal brain using rapid-scanning x-ray fluorescence. Phys Med Biol (2009) 1.11
Structures of the cuprous-thiolate clusters of the Mac1 and Ace1 transcriptional activators. Biochemistry (2002) 1.09
Modified active site coordination in a clinical mutant of sulfite oxidase. J Am Chem Soc (2007) 1.09
Localizing the biochemical transformations of arsenate in a hyperaccumulating fern. Environ Sci Technol (2006) 1.09
Analysis of sulfur and selenium assimilation in Astragalus plants with varying capacities to accumulate selenium. Plant J (2005) 1.09
Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region. J Mol Biol (2005) 1.08
Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. Mol Cell Neurosci (2012) 1.08
Chemical form and distribution of selenium and sulfur in the selenium hyperaccumulator Astragalus bisulcatus. Plant Physiol (2003) 1.08
characterization of the cytochrome c oxidase assembly factor Cox19 of Saccharomyces cerevisiae. J Biol Chem (2007) 1.04
Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments. Mol Genet Metab (2007) 1.04
Electronic structure description of the cis-MoOS unit in models for molybdenum hydroxylases. J Am Chem Soc (2007) 1.03
Unraveling the substrate-metal binding site of ferrochelatase: an X-ray absorption spectroscopic study. Biochemistry (2002) 1.02
Production of Se-methylselenocysteine in transgenic plants expressing selenocysteine methyltransferase. BMC Plant Biol (2004) 1.01
Cryptic proteolytic activity of dihydrolipoamide dehydrogenase. Proc Natl Acad Sci U S A (2007) 1.01
The ferroxidase activity of yeast frataxin. J Biol Chem (2002) 1.01
Metalloprotein active site structure determination: synergy between X-ray absorption spectroscopy and X-ray crystallography. J Inorg Biochem (2012) 0.99
Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer. Biochemistry (2008) 0.98
Imaging of selenium in plants using tapered metal monocapillary optics. J Synchrotron Radiat (2003) 0.96
Chemical forms of mercury and selenium in fish following digestion with simulated gastric fluid. Chem Res Toxicol (2008) 0.96
Tetrathiomolybdate causes formation of hepatic copper-molybdenum clusters in an animal model of Wilson's disease. J Am Chem Soc (2003) 0.95
Mercury binding to the chelation therapy agents DMSA and DMPS and the rational design of custom chelators for mercury. Chem Res Toxicol (2004) 0.95
Recombinant Rhodobacter capsulatus xanthine dehydrogenase, a useful model system for the characterization of protein variants leading to xanthinuria I in humans. J Biol Chem (2003) 0.95
Localizing organomercury uptake and accumulation in zebrafish larvae at the tissue and cellular level. Proc Natl Acad Sci U S A (2008) 0.95
X-ray-induced photo-chemistry and X-ray absorption spectroscopy of biological samples. J Synchrotron Radiat (2012) 0.93
Elemental and chemically specific X-ray fluorescence imaging of biological systems. Chem Rev (2014) 0.93
High-resolution X-ray emission spectroscopy of molybdenum compounds. Inorg Chem (2005) 0.92
Active-site dynamics and large-scale domain motions of sulfite oxidase: a molecular dynamics study. J Phys Chem B (2010) 0.92
The active site of arsenite oxidase from Alcaligenes faecalis. J Am Chem Soc (2002) 0.92
Selenium accumulation in flowers and its effects on pollination. New Phytol (2011) 0.92
Partial conservation of functions between eukaryotic frataxin and the Escherichia coli frataxin homolog CyaY. FEMS Yeast Res (2007) 0.92
Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level. J Biol Chem (2008) 0.91
The characterization and role of zinc binding in yeast Cox4. J Biol Chem (2007) 0.91
The chemical form of mitochondrial iron in Friedreich's ataxia. J Inorg Biochem (2007) 0.90
The chemical nature of mercury in human brain following poisoning or environmental exposure. ACS Chem Neurosci (2010) 0.90
Nature of the catalytically labile oxygen at the active site of xanthine oxidase. J Am Chem Soc (2005) 0.90
Biliary excretion of [(GS)(2)AsSe](-) after intravenous injection of rabbits with arsenite and selenate. Chem Res Toxicol (2002) 0.90
Copper sensing function of Drosophila metal-responsive transcription factor-1 is mediated by a tetranuclear Cu(I) cluster. Nucleic Acids Res (2008) 0.89
Using softer X-ray absorption spectroscopy to probe biological systems. J Synchrotron Radiat (2005) 0.89