Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes

Non-native Soluble Oligomers of Cu/Zn Superoxide Dismutase (SOD1) Contain a Conformational Epitope Linked to Cytotoxicity in Amyotrophic Lateral Sclerosis (ALS)

(2014) Rachel L. Redler et al.   BIOCHEMISTRY

Copper-Based Pulsed Dipolar ESR Spectroscopy as a Probe of Protein Conformation Linked to Disease States

(2014) Gregory E. Merz et al.   BIOPHYSICAL JOURNAL

Many roads lead to Rome? Multiple modes of Cu,Zn superoxide dismutase destabilization, misfolding and aggregation in amyotrophic lateral sclerosis

(2014) Helen R. Broom et al.   Essays in Biochemistry

Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

(2014) B. R. Roberts et al.   JOURNAL OF NEUROSCIENCE

Super-Resolution in Solution X-Ray Scattering and Its Applications to Structural Systems Biology

(2013) Robert P. Rambo et al.   Annual Review of Biophysics

Implementation and performance of SIBYLS: a dual endstation small-angle X-ray scattering and macromolecular crystallography beamline at the Advanced Light Source

(2013) Scott Classen et al.   JOURNAL OF APPLIED CRYSTALLOGRAPHY

Comprehensive macromolecular conformations mapped by quantitative SAXS analyses

(2013) Greg L Hura et al.   NATURE METHODS

Identification of a novel missense (C7W) mutation of SOD1 in a large familial amyotrophic lateral sclerosis pedigree

(2013) Zhanjun Wang et al.   NEUROBIOLOGY OF AGING

Marked intrafamilial phenotypic variation in a family with SOD1 C111Y mutation

(2012) Akinori Nakamura et al.   Amyotrophic Lateral Sclerosis

ALSoD: A user-friendly online bioinformatics tool for amyotrophic lateral sclerosis genetics

(2012) Olubunmi Abel et al.   HUMAN MUTATION

Early Steps in Oxidation-Induced SOD1 Misfolding: Implications for Non-Amyloid Protein Aggregation in Familial ALS

(2012) Vikram Khipple Mulligan et al.   JOURNAL OF MOLECULAR BIOLOGY

Degenerative myelopathy associated with a missense mutation in the superoxide dismutase 1 (SOD1) gene progresses to peripheral neuropathy in Pembroke Welsh Corgis and Boxers

(2012) G. Diane Shelton et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Oxidative Modification of Cysteine 111 Promotes Disulfide Bond-Independent Aggregation of SOD1

(2012) Xueping Chen et al.   NEUROCHEMICAL RESEARCH

An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

(2012) S. Guareschi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Glutathionylation at Cys-111 Induces Dissociation of Wild Type and FALS Mutant SOD1 Dimers

(2011) Rachel L. Redler et al.   BIOCHEMISTRY

Characterizing flexible and intrinsically unstructured biological macromolecules by SAS using the Porod-Debye law

(2011) Robert P. Rambo et al.   BIOPOLYMERS

Metals in biology: defining metalloproteomes

(2011) Steven M Yannone et al.   CURRENT OPINION IN BIOTECHNOLOGY

XRCC4 Protein Interactions with XRCC4-like Factor (XLF) Create an Extended Grooved Scaffold for DNA Ligation and Double Strand Break Repair

(2011) Michal Hammel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ALS-Causing SOD1 Mutations Promote Production of Copper-Deficient Misfolded Species

(2011) Philbert Ip et al.   JOURNAL OF MOLECULAR BIOLOGY

Local Unfolding of Cu, Zn Superoxide Dismutase Monomer Determines the Morphology of Fibrillar Aggregates

(2011) Feng Ding et al.   JOURNAL OF MOLECULAR BIOLOGY

Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS

(2011) K. A. Vassall et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form

(2010) Aaron Kerman et al.   ACTA NEUROPATHOLOGICA

Structural dynamics in DNA damage signaling and repair

(2010) J Jefferson P Perry et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Bridging the solution divide: comprehensive structural analyses of dynamic RNA, DNA, and protein assemblies by small-angle X-ray scattering

(2010) Robert P Rambo et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

SOD1 Mutations Targeting Surface Hydrogen Bonds Promote Amyotrophic Lateral Sclerosis without Reducing Apo-state Stability

(2010) Roberth Byström et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Nonamyloid Aggregates Arising from Mature Copper/Zinc Superoxide Dismutases Resemble Those Observed in Amyotrophic Lateral Sclerosis

(2010) Young-Mi Hwang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Microbial metalloproteomes are largely uncharacterized

(2010) Aleksandar Cvetkovic et al.   NATURE

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

FoXS: a web server for rapid computation and fitting of SAXS profiles

(2010) D. Schneidman-Duhovny et al.   NUCLEIC ACIDS RESEARCH

The ALS-Associated Mutation G93A in Human Copper-Zinc Superoxide Dismutase Selectively Destabilizes the Remote Metal Binding Region

(2009) Anna Katrine Museth et al.   BIOCHEMISTRY

The structural biochemistry of the superoxide dismutases

(2009) J.J.P. Perry et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Assessment of long-term molecular dynamics calculations with experimental information on protein shape from X-ray scattering – SOD1 as a case study

(2009) C.W. Yong et al.   CHEMICAL PHYSICS LETTERS

Structure and flexibility within proteins as identified through small angle X-ray scattering

(2009) M. Pelikan et al.   GENERAL PHYSIOLOGY AND BIOPHYSICS

Variation in aggregation propensities among ALS-associated variants of SOD1: Correlation to human disease

(2009) Mercedes Prudencio et al.   HUMAN MOLECULAR GENETICS

Robust, high-throughput solution structural analyses by small angle X-ray scattering (SAXS)

(2009) Greg L Hura et al.   NATURE METHODS

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

(2009) Marjatta Son et al.   NEUROBIOLOGY OF DISEASE

Transient structural distortion of metal-free Cu/Zn superoxide dismutase triggers aberrant oligomerization

(2009) K. Teilum et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutants

(2009) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Genome-wide association analysis reveals aSOD1mutation in canine degenerative myelopathy that resembles amyotrophic lateral sclerosis

(2009) Tomoyuki Awano et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Limited Role for Disulfide Cross-linking in the Aggregation of Mutant SOD1 Linked to Familial Amyotrophic Lateral Sclerosis

(2008) Celeste M. Karch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Complete Loss of Post-translational Modifications Triggers Fibrillar Aggregation of SOD1 in the Familial Form of Amyotrophic Lateral Sclerosis

(2008) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small-Angle X-ray Scattering Reveals the N-Terminal Domain Organization of Cardiac Myosin Binding Protein C

(2008) Cy M. Jeffries et al.   JOURNAL OF MOLECULAR BIOLOGY

Superoxide Dismutase from the Eukaryotic Thermophile Alvinella pompejana: Structures, Stability, Mechanism, and Insights into Amyotrophic Lateral Sclerosis

(2008) David S. Shin et al.   JOURNAL OF MOLECULAR BIOLOGY

Initiation and elongation in fibrillation of ALS-linked superoxide dismutase

(2008) M. Chattopadhyay et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein Aggregation and Protein Instability Govern Familial Amyotrophic Lateral Sclerosis Patient Survival

(2008) Qi Wang et al.   PLOS BIOLOGY

Structure and dynamics of copper-free SOD: The protein before binding copper

(2002) Lucia Banci et al.   PROTEIN SCIENCE