Published in FEBS J on July 25, 2007
Iron-sulfur cluster biogenesis and human disease. Trends Genet (2008) 3.03
Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS. Nat Struct Mol Biol (2009) 2.08
Building Fe-S proteins: bacterial strategies. Nat Rev Microbiol (2010) 1.83
The pathogenesis of Friedreich ataxia and the structure and function of frataxin. J Neurol (2009) 1.68
Frataxin and mitochondrial FeS cluster biogenesis. J Biol Chem (2010) 1.49
The iron-sulfur cluster assembly machineries in plants: current knowledge and open questions. Front Plant Sci (2013) 1.13
A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model. Hum Mol Genet (2011) 0.94
Yeast frataxin is stabilized by low salt concentrations: cold denaturation disentangles ionic strength effects from specific interactions. PLoS One (2014) 0.91
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation. Front Pharmacol (2014) 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
Understanding the frustration arising from the competition between function, misfolding, and aggregation in a globular protein. Proc Natl Acad Sci U S A (2014) 0.86
Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism. PLoS Genet (2015) 0.80
The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization. J Biol Chem (2013) 0.80
Selective observation of the disordered import signal of a globular protein by in-cell NMR: the example of frataxins. Protein Sci (2015) 0.79
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
His86 from the N-terminus of frataxin coordinates iron and is required for Fe-S cluster synthesis. Biochemistry (2013) 0.78
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
A natural and readily available crowding agent: NMR studies of proteins in hen egg white. Proteins (2011) 0.76
On the Effect of Sodium Chloride and Sodium Sulfate on Cold Denaturation. PLoS One (2015) 0.75
New Techniques for Ancient Proteins: Direct Coupling Analysis Applied on Proteins Involved in Iron Sulfur Cluster Biogenesis. Front Mol Biosci (2017) 0.75
Structural characterization of metal binding to a cold-adapted frataxin. J Biol Inorg Chem (2015) 0.75
SAXS and stability studies of iron-induced oligomers of bacterial frataxin CyaY. PLoS One (2017) 0.75
EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers. Nat Struct Mol Biol (2008) 5.32
Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28. Nat Genet (2010) 2.66
Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS. Nat Struct Mol Biol (2009) 2.08
Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins. FEBS Lett (2002) 1.78
From Alzheimer to Huntington: why is a structural understanding so difficult? EMBO J (2003) 1.68
The pathogenesis of Friedreich ataxia and the structure and function of frataxin. J Neurol (2009) 1.68
The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition. Proc Natl Acad Sci U S A (2005) 1.62
Structural bases for the interaction of frataxin with the central components of iron-sulphur cluster assembly. Nat Commun (2010) 1.56
Solution structure of the bacterial frataxin ortholog, CyaY: mapping the iron binding sites. Structure (2004) 1.55
G-quartet-dependent recognition between the FMRP RGG box and RNA. RNA (2003) 1.54
The structure of the N-terminal domain of the fragile X mental retardation protein: a platform for protein-protein interaction. Structure (2006) 1.45
The structure of Lethocerus troponin C: insights into the mechanism of stretch activation in muscles. Structure (2007) 1.45
Iron binding and oxidation kinetics in frataxin CyaY of Escherichia coli. J Mol Biol (2004) 1.42
Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3. J Mol Biol (2004) 1.41
Polyglutamine is not all: the functional role of the AXH domain in the ataxin-1 protein. J Mol Biol (2005) 1.36
Fragile X mental retardation protein (FMRP) binds specifically to the brain cytoplasmic RNAs BC1/BC200 via a novel RNA-binding motif. J Biol Chem (2005) 1.32
Pathogenic and non-pathogenic polyglutamine tracts have similar structural properties: towards a length-dependent toxicity gradient. J Mol Biol (2007) 1.31
SCA3: neurological features, pathogenesis and animal models. Cerebellum (2008) 1.31
Structural basis for increased toxicity of pathological aβ42:aβ40 ratios in Alzheimer disease. J Biol Chem (2011) 1.26
Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly. EMBO Rep (2006) 1.24
Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis. Biochemistry (2012) 1.19
Crystal structure of the alcohol dehydrogenase from the hyperthermophilic archaeon Sulfolobus solfataricus at 1.85 A resolution. J Mol Biol (2002) 1.17
Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites. Biopolymers (2009) 1.17
Domain architecture of the polyglutamine protein ataxin-3: a globular domain followed by a flexible tail. FEBS Lett (2003) 1.16
The C terminus of fragile X mental retardation protein interacts with the multi-domain Ran-binding protein in the microtubule-organising centre. J Mol Biol (2004) 1.15
The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils. PLoS One (2006) 1.15
Functional interactions as a survival strategy against abnormal aggregation. FASEB J (2010) 1.13
The factors governing the thermal stability of frataxin orthologues: how to increase a protein's stability. Biochemistry (2004) 1.13
Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117. J Biol Chem (2010) 1.12
Protein stability in nanocages: a novel approach for influencing protein stability by molecular confinement. J Mol Biol (2004) 1.12
Clinical and genetic aspects of Bernard-Soulier syndrome: searching for genotype/phenotype correlations. Haematologica (2010) 1.11
Interaction of sweet proteins with their receptor. A conformational study of peptides corresponding to loops of brazzein, monellin and thaumatin. Eur J Biochem (2004) 1.11
Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factors. PLoS One (2009) 1.11
Y65C missense mutation in the WW domain of the Golabi-Ito-Hall syndrome protein PQBP1 affects its binding activity and deregulates pre-mRNA splicing. J Biol Chem (2010) 1.09
Thrombin-aptamer recognition: a revealed ambiguity. Nucleic Acids Res (2011) 1.06
Crystal structure of a ternary complex of the alcohol dehydrogenase from Sulfolobus solfataricus. Biochemistry (2003) 1.06
Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding. Biochem J (2006) 1.05
High-resolution structures of two complexes between thrombin and thrombin-binding aptamer shed light on the role of cations in the aptamer inhibitory activity. Nucleic Acids Res (2012) 1.04
Bacterial IscU is a well folded and functional single domain protein. Eur J Biochem (2004) 1.02
Of the vulnerability of orphan complex proteins: the case study of the E. coli IscU and IscS proteins. Protein Expr Purif (2010) 1.02
Role of dimerization in KH/RNA complexes: the example of Nova KH3. Biochemistry (2002) 1.00
MYH9-related disease: five novel mutations expanding the spectrum of causative mutations and confirming genotype/phenotype correlations. Eur J Med Genet (2012) 1.00
Prion fibrillization is mediated by a native structural element that comprises helices H2 and H3. J Biol Chem (2010) 1.00
Solution structure of ZASP PDZ domain; implications for sarcomere ultrastructure and enigma family redundancy. Structure (2004) 0.99
Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One (2010) 0.99
82-FIP, a novel FMRP (fragile X mental retardation protein) interacting protein, shows a cell cycle-dependent intracellular localization. Hum Mol Genet (2003) 0.99
A standardized and biocompatible preparation of aggregate-free amyloid beta peptide for biophysical and biological studies of Alzheimer's disease. Protein Eng Des Sel (2011) 0.99
Cold denaturation of yeast frataxin offers the clue to understand the effect of alcohols on protein stability. J Am Chem Soc (2008) 0.98
Regulating the contraction of insect flight muscle. J Muscle Res Cell Motil (2011) 0.98
Spectrum of the mutations in Bernard-Soulier syndrome. Hum Mutat (2014) 0.97
Increasing the X-ray diffraction power of protein crystals by dehydration: the case of bovine serum albumin and a survey of literature data. Int J Mol Sci (2012) 0.97
The role of interruptions in polyQ in the pathology of SCA1. PLoS Genet (2013) 0.96
Ferredoxin competes with bacterial frataxin in binding to the desulfurase IscS. J Biol Chem (2013) 0.96
YfhJ, a molecular adaptor in iron-sulfur cluster formation or a frataxin-like protein? Structure (2006) 0.95
The role of CyaY in iron sulfur cluster assembly on the E. coli IscU scaffold protein. PLoS One (2011) 0.95
Structure validation of the Josephin domain of ataxin-3: conclusive evidence for an open conformation. J Biomol NMR (2006) 0.94
Dynamics, stability and iron-binding activity of frataxin clinical mutants. FEBS J (2008) 0.93
The AXH domain adopts alternative folds the solution structure of HBP1 AXH. Structure (2005) 0.93
The role of zinc in the stability of the marginally stable IscU scaffold protein. Protein Sci (2014) 0.92
Self-assembly and conformational heterogeneity of the AXH domain of ataxin-1: an unusual example of a chameleon fold. Biophys J (2013) 0.92
Understanding cold denaturation: the case study of Yfh1. J Am Chem Soc (2010) 0.92
The importance of dynamic effects on the enzyme activity: X-ray structure and molecular dynamics of onconase mutants. J Biol Chem (2005) 0.91
The N-terminus of mature human frataxin is intrinsically unfolded. FEBS J (2009) 0.90
Assignment of the 1H, 13C, and 15N resonances of the Josephin domain of human ataxin-3. J Biomol NMR (2004) 0.90
Using dynamics-based comparisons to predict nucleic acid binding sites in proteins: an application to OB-fold domains. Bioinformatics (2009) 0.88
A study of the ultrastructure of fragile-X-related proteins. Biochem J (2009) 0.88
The Josephin domain determines the morphological and mechanical properties of ataxin-3 fibrils. Biophys J (2011) 0.87
A major IgE epitope-containing grass pollen allergen domain from Phl p 5 folds as a four-helix bundle. Protein Eng (2002) 0.86
Mechanical properties of the domains of titin in a Go-like model. J Chem Phys (2005) 0.86
Structural study of a single-point mutant of Sulfolobus solfataricus alcohol dehydrogenase with enhanced activity. FEBS Lett (2003) 0.86
3'-Phosphoadenosine 5'-phosphosulfate (PAPS) synthases, naturally fragile enzymes specifically stabilized by nucleotide binding. J Biol Chem (2012) 0.85
The role of unstructured extensions in the rotational diffusion properties of a globular protein: the example of the titin i27 module. Biophys J (2004) 0.84
Ab initio study of NMR 15N chemical shift differences induced by Ca2+ binding to EF-hand proteins. Biochemistry (2004) 0.84
A S-adenosylmethionine methyltransferase-like domain within the essential, Fe-S-containing yeast protein Dre2. FEBS J (2012) 0.84
Solution model of the intrinsically disordered polyglutamine tract-binding protein-1. Biophys J (2012) 0.84
Structure and flexibility in cold-adapted iron superoxide dismutases: the case of the enzyme isolated from Pseudoalteromonas haloplanktis. J Struct Biol (2010) 0.84
The role of a clinically important mutation in the fold and RNA-binding properties of KH motifs. RNA (2003) 0.84
The buried diversity of bovine seminal ribonuclease: shape and cytotoxicity of the swapped non-covalent form of the enzyme. J Mol Biol (2007) 0.83
Structure of the C-terminal domain of Neisseria heparin binding antigen (NHBA), one of the main antigens of a novel vaccine against Neisseria meningitidis. J Biol Chem (2011) 0.83
Duplex-quadruplex motifs in a peculiar structural organization cooperatively contribute to thrombin binding of a DNA aptamer. Acta Crystallogr D Biol Crystallogr (2013) 0.83
The SH3 domain of nebulin binds selectively to type II peptides: theoretical prediction and experimental validation. J Mol Biol (2002) 0.83
Conformational plasticity of proNGF. PLoS One (2011) 0.83
A novel ErbB2 epitope targeted by human antitumor immunoagents. FEBS J (2011) 0.82
Probing the activity of diguanylate cyclases and c-di-GMP phosphodiesterases in real-time by CD spectroscopy. Nucleic Acids Res (2013) 0.82
Integrity of helix 2-helix 3 domain of the PrP protein is not mandatory for prion replication. J Biol Chem (2012) 0.82
Correlation between Raman and X-ray crystallography data of (Pro-Pro-Gly)10. Biophys Chem (2008) 0.82
Structural characterization of ferric hemoglobins from three antarctic fish species of the suborder notothenioidei. Biophys J (2007) 0.82
Population shift vs induced fit: the case of bovine seminal ribonuclease swapping dimer. Biopolymers (2004) 0.82
MYH9 related disease: a novel missense Ala95Asp mutation of the MYH9 gene. Platelets (2009) 0.82
Crystallization and preliminary X-ray analysis of the complex of human alpha-thrombin with a modified thrombin-binding aptamer. Acta Crystallogr Sect F Struct Biol Cryst Commun (2010) 0.81
NMR and molecular dynamics studies of the interaction of melatonin with calmodulin. Protein Sci (2004) 0.81
A new RNase sheds light on the RNase/angiogenin subfamily from zebrafish. Biochem J (2011) 0.81
WHEATSHEAF: an algorithm to average protein structure ensembles. Acta Crystallogr D Biol Crystallogr (2004) 0.81
Cold denaturation and aggregation: a comparative NMR study of titin I28 in bulk and in a confined environment. J Am Chem Soc (2009) 0.81
Solution structure of the Apo C-terminal domain of the Lethocerus F1 troponin C isoform. Biochemistry (2010) 0.81
The role of the hinge loop in domain swapping. The special case of bovine seminal ribonuclease. J Biol Chem (2005) 0.81
Correlation between hemichrome stability and the root effect in tetrameric hemoglobins. Biophys J (2009) 0.81