Prion-like activity of Cu/Zn superoxide dismutase

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

(2014) Leslie I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) Yoshiaki Furukawa et al.   FEBS LETTERS

Mutant Copper-Zinc Superoxide Dismutase (SOD1) Induces Protein Secretion Pathway Alterations and Exosome Release in Astrocytes

(2013) Manuela Basso et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Wild-type Cu/Zn superoxide dismutase stabilizes mutant variants by heterodimerization

(2013) Anna Weichert et al.   NEUROBIOLOGY OF DISEASE

Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

(2013) Takashi Nonaka et al.   Cell Reports

Evaluation of Potential Infectivity of Alzheimer and Parkinson Disease Proteins in Recipients of Cadaver-Derived Human Growth Hormone

(2013) David J. Irwin et al.   JAMA Neurology

Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p

(2012) Heather Stewart et al.   ACTA NEUROPATHOLOGICA

Sustained levels of antibodies against Aβ in amyloid-rich regions of the CNS following intravenous dosing in human APP transgenic mice

(2012) Frédérique Bard et al.   EXPERIMENTAL NEUROLOGY

Prion-infected cells regulate the release of exosomes with distinct ultrastructural features

(2012) Bradley M. Coleman et al.   FASEB JOURNAL

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

(2012) Matthis Synofzik et al.   HUMAN MOLECULAR GENETICS

The Exosome Secretory Pathway Transports Amyloid Precursor Protein Carboxyl-terminal Fragments from the Cell into the Brain Extracellular Space

(2012) Rocio Perez-Gonzalez et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Exosomal cell-to-cell transmission of alpha synuclein oligomers

(2012) Karin M Danzer et al.   Molecular Neurodegeneration

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

Aberrant Localization of FUS and TDP43 Is Associated with Misfolding of SOD1 in Amyotrophic Lateral Sclerosis

(2012) Edward Pokrishevsky et al.   PLoS One

Pathological  -Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice

(2012) K. C. Luk et al.   SCIENCE

A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions

(2011) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Toward a Mechanism of Prion Misfolding and Structural Models of PrPSc: Current Knowledge and Future Directions

(2011) Will C. Guest et al.   JOURNAL OF TOXICOLOGY AND ENVIRONMENTAL HEALTH-PART A-CURRENT ISSUES

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

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

Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: Implication for protein aggregation and immune response

(2011) Caterina Bendotti et al.   PROGRESS IN NEUROBIOLOGY

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

Updates on amyotrophic lateral sclerosis: Improving patient care

(2010) Walter G. Bradley   ANNALS OF NEUROLOGY

An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1

(2010) Mercedes Prudencio et al.   HUMAN MOLECULAR GENETICS

Exposure of Hydrophobic Surfaces Initiates Aggregation of Diverse ALS-Causing Superoxide Dismutase-1 Mutants

(2010) Christian Münch et al.   JOURNAL OF MOLECULAR BIOLOGY

Cell-Produced  -Synuclein Is Secreted in a Calcium-Dependent Manner by Exosomes and Impacts Neuronal Survival

(2010) E. Emmanouilidou et al.   JOURNAL OF NEUROSCIENCE

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden 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

Wild-type human SOD1 overexpression does not accelerate motor neuron disease in mice expressing murine Sod1G86R

(2010) Jean-Nicolas Audet et al.   NEUROBIOLOGY OF DISEASE

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

Novel Antibodies Reveal Inclusions Containing Non-Native SOD1 in Sporadic ALS Patients

(2010) Karin Forsberg et al.   PLoS One

Peripherally Applied A -Containing Inoculates Induce Cerebral  -Amyloidosis

(2010) Y. S. Eisele et al.   SCIENCE

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

(2009) Mercedes Prudencio et al.   HUMAN MOLECULAR GENETICS

Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse

(2009) L. Wang et al.   HUMAN MOLECULAR GENETICS

Wild-type Cu/Zn superoxide dismutase (SOD1) does not facilitate, but impedes the formation of protein aggregates of amyotrophic lateral sclerosis causing mutant SOD1

(2009) Heidrun Witan et al.   NEUROBIOLOGY OF DISEASE

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

(2009) J. M. Ravits et al.   NEUROLOGY

The Transcellular Spread of Cytosolic Amyloids, Prions, and Prionoids

(2009) Adriano Aguzzi et al.   NEURON

Induction of cerebral  -amyloidosis: Intracerebral versus systemic A  inoculation

(2009) Y. S. Eisele et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exogenous  -synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells

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

50bp deletion in the promoter for superoxide dismutase 1 (SOD1) reduces SOD1 expression in vitro and may correlate with increased age of onset of sporadic amyotrophic lateral sclerosis

(2008) Wendy J. Broom et al.   Amyotrophic Lateral Sclerosis

Superoxide dismutase: The cause of all amyotrophic lateral sclerosis?

(2008) Jorge Matias-Guiu et al.   ANNALS OF NEUROLOGY

The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS

(2008) Makoto Urushitani et al.   FASEB JOURNAL

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

Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme

(2008) Mercedes Prudencio et al.   JOURNAL OF NEUROCHEMISTRY

Amyloid-β protein dimers isolated directly from Alzheimer's brains impair synaptic plasticity and memory

(2008) Ganesh M Shankar et al.   NATURE MEDICINE

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

(2008) Qi Wang et al.   PLOS BIOLOGY