Published in Biopolymers on December 01, 2009
Breaking the chains: structure and function of the deubiquitinases. Nat Rev Mol Cell Biol (2009) 8.85
Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3. Brain (2009) 1.66
Toward understanding Machado-Joseph disease. Prog Neurobiol (2011) 1.42
Balancing act: deubiquitinating enzymes in the nervous system. Trends Neurosci (2011) 1.17
Functional interactions as a survival strategy against abnormal aggregation. FASEB J (2010) 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
Small heat-shock proteins interact with a flanking domain to suppress polyglutamine aggregation. Proc Natl Acad Sci U S A (2010) 1.11
PTMs in conversation: activity and function of deubiquitinating enzymes regulated via post-translational modifications. Cell Biochem Biophys (2011) 1.00
Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One (2010) 0.99
Crystal structure of a Josephin-ubiquitin complex: evolutionary restraints on ataxin-3 deubiquitinating activity. J Biol Chem (2010) 0.98
Energy landscapes of functional proteins are inherently risky. Nat Chem Biol (2014) 0.92
Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. Nat Commun (2014) 0.90
Trinucleotide repeats: a structural perspective. Front Neurol (2013) 0.89
The Josephin domain determines the morphological and mechanical properties of ataxin-3 fibrils. Biophys J (2011) 0.87
Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: implication in a loss-of-function pathology. Sci Rep (2014) 0.83
The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster. Neurobiol Dis (2015) 0.82
Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies. Mol Neurobiol (2013) 0.81
Examination of Ataxin-3 (atx-3) Aggregation by Structural Mass Spectrometry Techniques: A Rationale for Expedited Aggregation upon Polyglutamine (polyQ) Expansion. Mol Cell Proteomics (2015) 0.81
Characterization of the conformational fluctuations in the Josephin domain of ataxin-3. Biophys J (2014) 0.79
Enhanced Molecular Mobility of Ordinarily Structured Regions Drives Polyglutamine Disease. J Biol Chem (2015) 0.78
Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models. J Cell Biol (2016) 0.77
Investigation of the Josephin Domain protein-protein interaction by molecular dynamics. PLoS One (2014) 0.77
MJD and OTU deubiquitinating enzymes in Schistosoma mansoni. Parasitol Res (2015) 0.76
Josephin Domain Structural Conformations Explored by Metadynamics in Essential Coordinates. PLoS Comput Biol (2016) 0.76
Ataxin-3 expression correlates with the clinicopathologic features of gastric cancer. Int J Clin Exp Med (2014) 0.76
Thermodynamic and kinetic stability of the Josephin Domain closed arrangement: evidences from replica exchange molecular dynamics. Biol Direct (2017) 0.75
Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch. Front Neurosci (2017) 0.75
Allosteric regulation of deubiquitylase activity through ubiquitination. Front Mol Biosci (2015) 0.75
EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers. Nat Struct Mol Biol (2008) 5.32
Mutation of the RAD51C gene in a Fanconi anemia-like disorder. Nat Genet (2010) 3.57
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
Determination of secondary structure populations in disordered states of proteins using nuclear magnetic resonance chemical shifts. Biochemistry (2012) 1.87
RNA-dependent oligomerization of APOBEC3G is required for restriction of HIV-1. PLoS Pathog (2009) 1.84
cAMP activation of PKA defines an ancient signaling mechanism. Proc Natl Acad Sci U S A (2006) 1.79
Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins. FEBS Lett (2002) 1.78
Atomic structure and hierarchical assembly of a cross-β amyloid fibril. Proc Natl Acad Sci U S A (2013) 1.75
MicroRNA-199b-5p impairs cancer stem cells through negative regulation of HES1 in medulloblastoma. PLoS One (2009) 1.73
The alpha-to-beta conformational transition of Alzheimer's Abeta-(1-42) peptide in aqueous media is reversible: a step by step conformational analysis suggests the location of beta conformation seeding. Chembiochem (2006) 1.70
POPS: A fast algorithm for solvent accessible surface areas at atomic and residue level. Nucleic Acids Res (2003) 1.69
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 C-terminal KH domains of KSRP reveals a noncanonical motif important for mRNA degradation. Structure (2007) 1.49
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
Detection of allosteric signal transmission by information-theoretic analysis of protein dynamics. FASEB J (2011) 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
Release of high-energy water as an essential driving force for the high-affinity binding of cucurbit[n]urils. J Am Chem Soc (2012) 1.31
Prion and water: tight and dynamical hydration sites have a key role in structural stability. Proc Natl Acad Sci U S A (2005) 1.30
B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease. PLoS One (2012) 1.29
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
Understanding the molecular basis for the inhibition of the Alzheimer's Abeta-peptide oligomerization by human serum albumin using saturation transfer difference and off-resonance relaxation NMR spectroscopy. J Am Chem Soc (2007) 1.23
Characterization of the conformational equilibrium between the two major substates of RNase A using NMR chemical shifts. J Am Chem Soc (2012) 1.20
Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis. Biochemistry (2012) 1.19
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
Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1. Hum Mol Genet (2007) 1.12
Clinical and genetic aspects of Bernard-Soulier syndrome: searching for genotype/phenotype correlations. Haematologica (2010) 1.11
Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factors. PLoS One (2009) 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
Understanding the binding properties of an unusual metal-binding protein--a study of bacterial frataxin. FEBS J (2007) 1.10
Structural characterization of a misfolded intermediate populated during the folding process of a PDZ domain. Nat Struct Mol Biol (2010) 1.10
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
The oligomerization properties of prion protein are restricted to the H2H3 domain. FASEB J (2010) 1.08
Hydrogen-bonding propensities of sphingomyelin in solution and in a bilayer assembly: a molecular dynamics study. Biophys J (2003) 1.08
Plasticity of influenza haemagglutinin fusion peptides and their interaction with lipid bilayers. Biophys J (2004) 1.06
Conformational stability of human frataxin and effect of Friedreich's ataxia-related mutations on protein folding. Biochem J (2006) 1.05
A targeted siRNA screen identifies regulators of Cdc42 activity at the natural killer cell immunological synapse. Sci Signal (2011) 1.05
A new modified thrombin binding aptamer containing a 5'-5' inversion of polarity site. Nucleic Acids Res (2006) 1.04
Molecular interactions of ASPP1 and ASPP2 with the p53 protein family and the apoptotic promoters PUMA and Bax. Nucleic Acids Res (2008) 1.03
Correlation between omega and psi dihedral angles in protein structures. J Mol Biol (2005) 1.03
Of the vulnerability of orphan complex proteins: the case study of the E. coli IscU and IscS proteins. Protein Expr Purif (2010) 1.02
Bacterial IscU is a well folded and functional single domain protein. Eur J Biochem (2004) 1.02
POPSCOMP: an automated interaction analysis of biomolecular complexes. Nucleic Acids Res (2005) 1.00
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
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
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
Cold denaturation of yeast frataxin offers the clue to understand the effect of alcohols on protein stability. J Am Chem Soc (2008) 0.98
Glucokinase gene mutations: structural and genotype-phenotype analyses in MODY children from South Italy. PLoS One (2008) 0.98
How Förster resonance energy transfer imaging improves the understanding of protein interaction networks in cancer biology. Chemphyschem (2011) 0.98
Stability and structure of telomeric DNA sequences forming quadruplexes containing four G-tetrads with different topological arrangements. Biochemistry (2004) 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
Force-field dependence of chignolin folding and misfolding: comparison with experiment and redesign. Biophys J (2012) 0.97
The role of interruptions in polyQ in the pathology of SCA1. PLoS Genet (2013) 0.96
Determination of Conformational Equilibria in Proteins Using Residual Dipolar Couplings. J Chem Theory Comput (2011) 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
MinSet: a general approach to derive maximally representative database subsets by using fragment dictionaries and its application to the SCOP database. Bioinformatics (2007) 0.95
Large-scale modelling of the divergent spectrin repeats in nesprins: giant modular proteins. PLoS One (2013) 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
Stability of single sheet GNNQQNY aggregates analyzed by replica exchange molecular dynamics: antiparallel versus parallel association. Biochem Biophys Res Commun (2008) 0.93
The AXH domain adopts alternative folds the solution structure of HBP1 AXH. Structure (2005) 0.93