The Disulfide Bond, but Not Zinc or Dimerization, Controls Initiation and Seeded Growth in Amyotrophic Lateral Sclerosis-linked Cu,Zn Superoxide Dismutase (SOD1) Fibrillation

Curcumin binds to the pre-fibrillar aggregates of Cu/Zn superoxide dismutase (SOD1) and alters its amyloidogenic pathway resulting in reduced cytotoxicity

(2015) Nidhi K. Bhatia et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Targeting protein aggregation for the treatment of degenerative diseases

(2015) Yvonne S. Eisele et al.   NATURE REVIEWS DRUG DISCOVERY

Experimental transmissibility of mutant SOD1 motor neuron disease

(2014) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice

(2014) Guilian Xu et al.   HUMAN MOLECULAR GENETICS

In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants

(2014) Enrico Luchinat et al.   Nature Communications

SOD1 Aggregation and ALS: Role of Metallation States and Disulfide Status

(2013) Yuewei Sheng et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis

(2013) Yoshiaki Furukawa et al.   FEBS LETTERS

Atomic-resolution monitoring of protein maturation in live human cells by NMR

(2013) Lucia Banci et al.   Nature Chemical Biology

Disulfide Scrambling in Superoxide Dismutase 1 Reduces Its Cytotoxic Effect in Cultured Cells and Promotes Protein Aggregation

(2013) Lina Leinartaitė et al.   PLoS One

Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy

(2013) P. K. Chan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ligand binding and aggregation of pathogenic SOD1

(2013) Gareth S.A. Wright et al.   Nature Communications

The Amyloid State of Proteins in Human Diseases

(2012) David Eisenberg et al.   CELL

Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS

(2012) Matthis Synofzik et al.   HUMAN MOLECULAR GENETICS

Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis

(2012) K. S. Graffmo et al.   HUMAN MOLECULAR GENETICS

Disulfide Scrambling Describes the Oligomer Formation of Superoxide Dismutase (SOD1) Proteins in the Familial Form of Amyotrophic Lateral Sclerosis

(2012) Keisuke Toichi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Unifying Role for Prions in Neurodegenerative Diseases

(2012) S. B. Prusiner   SCIENCE

In-cell NMR in E. coli to Monitor Maturation Steps of hSOD1

(2011) Lucia Banci et al.   PLoS One

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

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

(2011) L. I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

(2011) Christian Münch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Copper and Zinc Metallation Status of Copper-Zinc Superoxide Dismutase from Amyotrophic Lateral Sclerosis Transgenic Mice

(2010) Herman L. Lelie et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Folding Catalysis by Transient Coordination of Zn2+to the Cu Ligands of the ALS-Associated Enzyme Cu/Zn Superoxide Dismutase 1

(2010) Lina Leinartaitė et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Metal-Free ALS Variants of Dimeric Human Cu,Zn-Superoxide Dismutase Have Enhanced Populations of Monomeric Species

(2010) Anna-Karin E. Svensson et al.   PLoS One

Superoxide Dismutase 1 and tgSOD1G93A Mouse Spinal Cord Seed Fibrils, Suggesting a Propagative Cell Death Mechanism in Amyotrophic Lateral Sclerosis

(2010) Ruth Chia et al.   PLoS One

Metal-free Superoxide Dismutase-1 and Three Different Amyotrophic Lateral Sclerosis Variants Share a Similar Partially Unfolded β-Barrel at Physiological Temperature

(2009) Armando Durazo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Metal Deficiency Increases Aberrant Hydrophobicity of Mutant Superoxide Dismutases That Cause Amyotrophic Lateral Sclerosis

(2009) Ashutosh Tiwari et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activation of Cu,Zn-Superoxide Dismutase in the Absence of Oxygen and the Copper Chaperone CCS

(2009) Jeffry M. Leitch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Loss of Metal Ions, Disulfide Reduction and Mutations Related to Familial ALS Promote Formation of Amyloid-Like Aggregates from Superoxide Dismutase

(2009) Zeynep A. Oztug Durer et al.   PLoS One

Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS

(2009) C. M. Karch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An Analytical Solution to the Kinetics of Breakable Filament Assembly

(2009) T. P. J. Knowles et al.   SCIENCE

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

Zinc Binding Modulates the Entire Folding Free Energy Surface of Human Cu,Zn Superoxide Dismutase

(2008) Can Kayatekin et al.   JOURNAL OF MOLECULAR BIOLOGY

Denaturational Stress Induces Formation of Zinc-Deficient Monomers of Cu,Zn Superoxide Dismutase: Implications for Pathogenesis in Amyotrophic Lateral Sclerosis

(2008) Vikram Khipple Mulligan et al.   JOURNAL OF MOLECULAR BIOLOGY

Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice

(2008) D. Jaarsma et al.   JOURNAL OF NEUROSCIENCE

Dynamical roles of metal ions and the disulfide bond in Cu, Zn superoxide dismutase folding and aggregation

(2008) F. Ding et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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