Published in J Mol Biol on November 22, 2002
Misfolded CuZnSOD and amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2003) 2.65
Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro. Proc Natl Acad Sci U S A (2003) 1.81
Dimer destabilization in superoxide dismutase may result in disease-causing properties: structures of motor neuron disease mutants. Proc Natl Acad Sci U S A (2004) 1.76
Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell (2014) 1.65
The structural biochemistry of the superoxide dismutases. Biochim Biophys Acta (2009) 1.50
ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes. BMC Neurosci (2003) 1.46
Superoxide dismutase from the eukaryotic thermophile Alvinella pompejana: structures, stability, mechanism, and insights into amyotrophic lateral sclerosis. J Mol Biol (2008) 1.32
Structural characterization of zinc-deficient human superoxide dismutase and implications for ALS. J Mol Biol (2007) 1.26
Rapid profiling of disease alleles using a tunable reporter of protein misfolding. Genetics (2012) 1.20
Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutants. Proc Natl Acad Sci U S A (2009) 1.12
Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg. Protein Sci (2005) 1.11
A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop. J Biol Chem (2009) 1.08
Contribution of human manganese superoxide dismutase tyrosine 34 to structure and catalysis. Biochemistry (2009) 0.93
Cu,Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability. J Biol Chem (2010) 0.90
A possible therapeutic target for Lou Gehrig's disease. Proc Natl Acad Sci U S A (2004) 0.89
Molecular characterization of two superoxide dismutases from Hydra vulgaris. Gene (2006) 0.87
Purification and crystallization of human Cu/Zn superoxide dismutase recombinantly produced in the protozoan Leishmania tarentolae. Acta Crystallogr Sect F Struct Biol Cryst Commun (2010) 0.85
Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes. Proc Natl Acad Sci U S A (2014) 0.80
Altered thiol chemistry in human amyotrophic lateral sclerosis-linked mutants of superoxide dismutase 1. J Biol Chem (2014) 0.78
Cis-suppression to arrest protein aggregation in mammalian cells. Biotechnol Bioeng (2013) 0.78
Cellular Redox Systems Impact the Aggregation of Cu,Zn Superoxide Dismutase Linked to Familial Amyotrophic Lateral Sclerosis. J Biol Chem (2016) 0.77
Structural switching of Cu,Zn-superoxide dismutases at loop VI: insights from the crystal structure of 2-mercaptoethanol-modified enzyme. Biosci Rep (2012) 0.77
Impact of genetic variation on three dimensional structure and function of proteins. PLoS One (2017) 0.75
X-ray solution scattering (SAXS) combined with crystallography and computation: defining accurate macromolecular structures, conformations and assemblies in solution. Q Rev Biophys (2007) 9.07
Robust, high-throughput solution structural analyses by small angle X-ray scattering (SAXS). Nat Methods (2009) 6.77
Structural mechanism of abscisic acid binding and signaling by dimeric PYR1. Science (2009) 4.41
Type IV pilus structure and bacterial pathogenicity. Nat Rev Microbiol (2004) 4.29
Mre11 dimers coordinate DNA end bridging and nuclease processing in double-strand-break repair. Cell (2008) 4.26
The Rad50 zinc-hook is a structure joining Mre11 complexes in DNA recombination and repair. Nature (2002) 3.48
Type IV pilus structure by cryo-electron microscopy and crystallography: implications for pilus assembly and functions. Mol Cell (2006) 3.36
Structural basis for FEN-1 substrate specificity and PCNA-mediated activation in DNA replication and repair. Cell (2004) 3.33
Accurate assessment of mass, models and resolution by small-angle scattering. Nature (2013) 3.18
XPD helicase structures and activities: insights into the cancer and aging phenotypes from XPD mutations. Cell (2008) 3.14
Mre11-Rad50-Nbs1 is a keystone complex connecting DNA repair machinery, double-strand break signaling, and the chromatin template. Biochem Cell Biol (2007) 3.13
Full-length archaeal Rad51 structure and mutants: mechanisms for RAD51 assembly and control by BRCA2. EMBO J (2003) 2.95
Nbs1 flexibly tethers Ctp1 and Mre11-Rad50 to coordinate DNA double-strand break processing and repair. Cell (2009) 2.90
Characterizing flexible and intrinsically unstructured biological macromolecules by SAS using the Porod-Debye law. Biopolymers (2011) 2.87
DNA binding and nucleotide flipping by the human DNA repair protein AGT. Nat Struct Mol Biol (2004) 2.86
Plant UVR8 photoreceptor senses UV-B by tryptophan-mediated disruption of cross-dimer salt bridges. Science (2012) 2.80
Type IV pilin structure and assembly: X-ray and EM analyses of Vibrio cholerae toxin-coregulated pilus and Pseudomonas aeruginosa PAK pilin. Mol Cell (2003) 2.78
SUMO-targeted ubiquitin ligases in genome stability. EMBO J (2007) 2.77
Microbial metalloproteomes are largely uncharacterized. Nature (2010) 2.62
Early abscisic acid signal transduction mechanisms: newly discovered components and newly emerging questions. Genes Dev (2010) 2.60
Bridging the solution divide: comprehensive structural analyses of dynamic RNA, DNA, and protein assemblies by small-angle X-ray scattering. Curr Opin Struct Biol (2010) 2.45
Human flap endonuclease structures, DNA double-base flipping, and a unified understanding of the FEN1 superfamily. Cell (2011) 2.44
ATM regulates Mre11-dependent DNA end-degradation and microhomology-mediated end joining. Cell Cycle (2010) 2.32
Structural basis for isozyme-specific regulation of electron transfer in nitric-oxide synthase. J Biol Chem (2004) 2.28
Human ABH3 structure and key residues for oxidative demethylation to reverse DNA/RNA damage. EMBO J (2006) 2.27
The intricate structural chemistry of base excision repair machinery: implications for DNA damage recognition, removal, and repair. DNA Repair (Amst) (2007) 2.26
A flexible interface between DNA ligase and PCNA supports conformational switching and efficient ligation of DNA. Mol Cell (2006) 2.23
DNA base damage recognition and removal: new twists and grooves. Mutat Res (2005) 2.21
The DNA repair endonuclease XPG interacts directly and functionally with the WRN helicase defective in Werner syndrome. Cell Cycle (2011) 2.15
Conserved XPB core structure and motifs for DNA unwinding: implications for pathway selection of transcription or excision repair. Mol Cell (2006) 2.13
Identification of a new cryptochrome class. Structure, function, and evolution. Mol Cell (2003) 2.11
A SIM-ultaneous role for SUMO and ubiquitin. Trends Biochem Sci (2008) 2.05
Science education. Educating future scientists. Science (2003) 1.97
Mre11-Rad50-Nbs1 conformations and the control of sensing, signaling, and effector responses at DNA double-strand breaks. DNA Repair (Amst) (2010) 1.96
Rad50/SMC proteins and ABC transporters: unifying concepts from high-resolution structures. Curr Opin Struct Biol (2003) 1.93
Ku and DNA-dependent protein kinase dynamic conformations and assembly regulate DNA binding and the initial non-homologous end joining complex. J Biol Chem (2009) 1.91
MDB: the Metalloprotein Database and Browser at The Scripps Research Institute. Nucleic Acids Res (2002) 1.89
Recognition of RNA polymerase II and transcription bubbles by XPG, CSB, and TFIIH: insights for transcription-coupled repair and Cockayne Syndrome. Mol Cell (2005) 1.89
Accurate SAXS profile computation and its assessment by contrast variation experiments. Biophys J (2013) 1.83
ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization. J Mol Biol (2003) 1.82
Hexameric structures of the archaeal secretion ATPase GspE and implications for a universal secretion mechanism. EMBO J (2007) 1.77
DNA charge transport as a first step in coordinating the detection of lesions by repair proteins. Proc Natl Acad Sci U S A (2012) 1.71
A structural model for regulation of NHEJ by DNA-PKcs autophosphorylation. DNA Repair (Amst) (2010) 1.71
Flipping of alkylated DNA damage bridges base and nucleotide excision repair. Nature (2009) 1.70
Implementation and performance of SIBYLS: a dual endstation small-angle X-ray scattering and macromolecular crystallography beamline at the Advanced Light Source. J Appl Crystallogr (2013) 1.69
DNA double-strand break repair from head to tail. Curr Opin Struct Biol (2002) 1.67
ABC ATPase signature helices in Rad50 link nucleotide state to Mre11 interface for DNA repair. Nat Struct Mol Biol (2011) 1.66
Nickel superoxide dismutase structure and mechanism. Biochemistry (2004) 1.66
Distinct dimer interaction and regulation in nitric-oxide synthase types I, II, and III. J Biol Chem (2002) 1.63
Improving small-angle X-ray scattering data for structural analyses of the RNA world. RNA (2010) 1.60
WRN exonuclease structure and molecular mechanism imply an editing role in DNA end processing. Nat Struct Mol Biol (2006) 1.55
Crystal structure of TM1367 from Thermotoga maritima at 1.90 A resolution reveals an atypical member of the cyclophilin (peptidylprolyl isomerase) fold. Proteins (2006) 1.51
XRCC4 protein interactions with XRCC4-like factor (XLF) create an extended grooved scaffold for DNA ligation and double strand break repair. J Biol Chem (2011) 1.48
Protein mimicry of DNA and pathway regulation. DNA Repair (Amst) (2005) 1.46
DNA apurinic-apyrimidinic site binding and excision by endonuclease IV. Nat Struct Mol Biol (2008) 1.46
XPB and XPD helicases in TFIIH orchestrate DNA duplex opening and damage verification to coordinate repair with transcription and cell cycle via CAK kinase. DNA Repair (Amst) (2011) 1.45
Mechanism and energetics of green fluorescent protein chromophore synthesis revealed by trapped intermediate structures. Proc Natl Acad Sci U S A (2003) 1.41
Tetrameric architecture of the circadian clock protein KaiB. A novel interface for intermolecular interactions and its impact on the circadian rhythm. J Biol Chem (2005) 1.39
Report of the wwPDB Small-Angle Scattering Task Force: data requirements for biomolecular modeling and the PDB. Structure (2013) 1.39
Biphasic coupling of neuronal nitric oxide synthase phosphorylation to the NMDA receptor regulates AMPA receptor trafficking and neuronal cell death. J Neurosci (2007) 1.38
Proliferating cell nuclear antigen loaded onto double-stranded DNA: dynamics, minor groove interactions and functional implications. Nucleic Acids Res (2006) 1.37
Structural basis for endothelial nitric oxide synthase binding to calmodulin. EMBO J (2003) 1.37
Functional motifs in the (6-4) photolyase crystal structure make a comparative framework for DNA repair photolyases and clock cryptochromes. Proc Natl Acad Sci U S A (2009) 1.36
Super-resolution in solution X-ray scattering and its applications to structural systems biology. Annu Rev Biophys (2013) 1.36
DNA binding, nucleotide flipping, and the helix-turn-helix motif in base repair by O6-alkylguanine-DNA alkyltransferase and its implications for cancer chemotherapy. DNA Repair (Amst) (2007) 1.36
XLF regulates filament architecture of the XRCC4·ligase IV complex. Structure (2010) 1.33
Superoxide dismutase from the eukaryotic thermophile Alvinella pompejana: structures, stability, mechanism, and insights into amyotrophic lateral sclerosis. J Mol Biol (2008) 1.32
Human DNA ligase III recognizes DNA ends by dynamic switching between two DNA-bound states. Biochemistry (2010) 1.32
Structural dynamics in DNA damage signaling and repair. Curr Opin Struct Biol (2010) 1.31
Cloning, expression, and characterization of a nitric oxide synthase protein from Deinococcus radiodurans. Proc Natl Acad Sci U S A (2001) 1.30
Comprehensive macromolecular conformations mapped by quantitative SAXS analyses. Nat Methods (2013) 1.29
gammaH2A binds Brc1 to maintain genome integrity during S-phase. EMBO J (2010) 1.28
Structures of endonuclease V with DNA reveal initiation of deaminated adenine repair. Nat Struct Mol Biol (2009) 1.26
Structural characterization of zinc-deficient human superoxide dismutase and implications for ALS. J Mol Biol (2007) 1.26
A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase. J Mol Biol (2006) 1.25
Anchored plasticity opens doors for selective inhibitor design in nitric oxide synthase. Nat Chem Biol (2008) 1.25
Direct observation of a photoinduced radical pair in a cryptochrome blue-light photoreceptor. Angew Chem Int Ed Engl (2009) 1.24
Surface charge interactions of the FMN module govern catalysis by nitric-oxide synthase. J Biol Chem (2006) 1.22
High-throughput SAXS for the characterization of biomolecules in solution: a practical approach. Methods Mol Biol (2014) 1.22
The C-terminal domain of yeast PCNA is required for physical and functional interactions with Cdc9 DNA ligase. Nucleic Acids Res (2007) 1.21
Structural analysis of flexible proteins in solution by small angle X-ray scattering combined with crystallography. J Struct Biol (2006) 1.21
Structural tuning of the fluorescent protein iLOV for improved photostability. J Biol Chem (2012) 1.19