A misfolded dimer of Cu/Zn-superoxide dismutase leading to pathological oligomerization in amyotrophic lateral sclerosis

Combined Isothermal Titration and Differential Scanning Calorimetry Define Three-State Thermodynamics of fALS-Associated Mutant Apo SOD1 Dimers and an Increased Population of Folded Monomer

(2016) Helen R. Broom et al.   BIOCHEMISTRY

Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis

(2015) Elizabeth A. Proctor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Destabilization of the dimer interface is a common consequence of diverse ALS-associated mutations in metal free SOD1

(2015) Helen R. Broom et al.   PROTEIN SCIENCE

Thermal fluctuations of immature SOD1 lead to separate folding and misfolding pathways

(2015) Ashok Sekhar et al.   eLife

A Primary Role for Disulfide Formation in the Productive Folding of Prokaryotic Cu,Zn-superoxide Dismutase

(2014) Yasuyuki Sakurai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2014) A. J. Pratt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2014) Enrico Luchinat et al.   Nature Communications

Early steps in thermal unfolding of superoxide dismutase 1 are similar to the conformational changes associated with the ALS-associated A4V mutation

(2013) T. Schmidlin et al.   PROTEIN ENGINEERING DESIGN & SELECTION

Ligand binding and aggregation of pathogenic SOD1

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

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

Quality control of protein standards for molecular mass determinations by small-angle X-ray scattering

(2010) Shuji Akiyama   JOURNAL OF APPLIED CRYSTALLOGRAPHY

Structural Changes to Monomeric CuZn Superoxide Dismutase Caused by the Familial Amyotrophic Lateral Sclerosis-Associated Mutation A4V

(2009) Tom Schmidlin et al.   BIOPHYSICAL JOURNAL

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

Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS

(2009) J. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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