Related references
Note: Only part of the references are listed.Variation in aggregation propensities among ALS-associated variants of SOD1: Correlation to human disease
Mercedes Prudencio et al.
HUMAN MOLECULAR GENETICS (2009)
A Common Property of Amyotrophic Lateral Sclerosis-associated Variants DESTABILIZATION OF THE COPPER/ZINC SUPEROXIDE DISMUTASE ELECTROSTATIC LOOP
Kathleen S. Molnar et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Transient structural distortion of metal-free Cu/Zn superoxide dismutase triggers aberrant oligomerization
Kaare Teilum et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity
Yoko Nekooki-Machida et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Destabilizing Protein Polymorphisms in the Genetic Background Direct Phenotypic Expression of Mutant SOD1 Toxicity
Tali Gidalevitz et al.
PLOS GENETICS (2009)
Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1
Bryan F. Shaw et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis
Yoshiaki Furukawa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS
Bradley J. Turner et al.
PROGRESS IN NEUROBIOLOGY (2008)
Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival
Qi Wang et al.
PLOS BIOLOGY (2008)
The coupling between disulphide status, metallation and dimer interface strength in Cu/Zn superoxide dismutase
Andreas Hornberg et al.
JOURNAL OF MOLECULAR BIOLOGY (2007)
Local unfolding in a destabilized, pathogenic variant of superoxide dismutase 1 observed with H/D exchange and mass spectrometry
Bryan Francis Shaw et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Onset and progression in inherited ALS determined by motor neurons and microglia
Severine Boillee et al.
SCIENCE (2006)
Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models
PA Jonsson et al.
BRAIN (2006)
Truncated wild-type SOD1 and FALS-linked mutant SOD1 cause neural cell death in the chick embryo spinal cord
GD Ghadge et al.
NEUROBIOLOGY OF DISEASE (2006)
Sequence and structural determinants of Cu, Zn superoxide dismutase aggregation
SD Khare et al.
PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS (2005)
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration:: αB-crystallin modulates aggregation
J Wang et al.
HUMAN MOLECULAR GENETICS (2005)
Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis
JA Rodriguez et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the Apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation
Y Furukawa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Fibril conformation as the basis of species- and strain-dependent seeding specificity of mammalian prion amyloids
EM Jones et al.
CELL (2005)
Conformational variations in an infectious protein determine prion strain differences
M Tanaka et al.
NATURE (2004)
Prediction of sequence-dependent and mutational effects on the aggregation of peptides and proteins
AM Fernandez-Escamilla et al.
NATURE BIOTECHNOLOGY (2004)
Oxygen-induced maturation of SOD1: a key role for disulfide formation by the copper chaperone CCS
Y Furukawa et al.
EMBO JOURNAL (2004)
Unraveling the mechanisms involved in motor neuron degeneration in ALS
LI Bruijn et al.
ANNUAL REVIEW OF NEUROSCIENCE (2004)
Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature
J Wang et al.
HUMAN MOLECULAR GENETICS (2003)
Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: Decreased stability of the apo state
MJ Lindberg et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site
J Wang et al.
NEUROBIOLOGY OF DISEASE (2002)
Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis
LJ Hayward et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues
M Watanabe et al.
NEUROBIOLOGY OF DISEASE (2001)
Neurodegenerative tauopathies
VMY Lee et al.
ANNUAL REVIEW OF NEUROSCIENCE (2001)