SOD1 Function and Its Implications for Amyotrophic Lateral Sclerosis Pathology

Experimental transmissibility of mutant SOD1 motor neuron disease

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

Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis

(2014) Melissa Nassif et al.   Autophagy

Modeling ALS with iPSCs Reveals that Mutant SOD1 Misregulates Neurofilament Balance in Motor Neurons

(2014) Hong Chen et al.   Cell Stem Cell

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

Prion-like activity of Cu/Zn superoxide dismutase

(2014) Leslie I Grad et al.   Prion

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

Genetic validation of a therapeutic target in a mouse model of ALS

(2014) A. S. de Boer et al.   Science Translational Medicine

Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress

(2014) Fabiola Rojas et al.   Frontiers in Cellular Neuroscience

Superoxide dismutase 1 acts as a nuclear transcription factor to regulate oxidative stress resistance

(2014) Chi Kwan Tsang et al.   Nature Communications

Establishing a novel C. elegans model to investigate the role of autophagy in amyotrophic lateral sclerosis

(2013) Jia Li et al.   ACTA PHARMACOLOGICA SINICA

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

(2013) Rachele A. Saccon et al.   BRAIN

SOD1 Integrates Signals from Oxygen and Glucose to Repress Respiration

(2013) Amit R. Reddi et al.   CELL

A Small Molecule Screen in Stem-Cell-Derived Motor Neurons Identifies a Kinase Inhibitor as a Candidate Therapeutic for ALS

(2013) Yin M. Yang et al.   Cell Stem Cell

Extracellular wildtype and mutant SOD1 induces ER–Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells

(2013) Vinod Sundaramoorthy et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

An enhanced integrated stress response ameliorates mutant SOD1-induced ALS

(2013) L. Wang et al.   HUMAN MOLECULAR GENETICS

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

Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis

(2013) Julie D. Atkin et al.   JOURNAL OF NEUROCHEMISTRY

Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability

(2013) Elsa Fritz et al.   JOURNAL OF NEUROPHYSIOLOGY

SOD1 as a Molecular Switch for Initiating the Homeostatic ER Stress Response under Zinc Deficiency

(2013) Kengo Homma et al.   MOLECULAR CELL

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models

(2013) Hyung-Jun Kim et al.   NATURE GENETICS

Exome sequencing to identify de novo mutations in sporadic ALS trios

(2013) Alessandra Chesi et al.   NATURE NEUROSCIENCE

Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival

(2013) Smita Saxena et al.   NEURON

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS

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

A novel monoclonal antibody reveals a conformational alteration shared by amyotrophic lateral sclerosis-linked SOD1 mutants

(2012) Takao Fujisawa et al.   ANNALS OF NEUROLOGY

Understanding ALS: new therapeutic approaches

(2012) Antonio Musarò   FEBS Journal

Autophagy and Its Comprehensive Impact on ALS

(2012) Cheng-yuan Song et al.   INTERNATIONAL JOURNAL OF NEUROSCIENCE

Prion-like spread of protein aggregates in neurodegeneration: Figure 1.

(2012) Magdalini Polymenidou et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Rapamycin treatment augments motor neuron degeneration in SOD1G93Amouse model of amyotrophic lateral sclerosis

(2011) Xiaojie Zhang et al.   Autophagy

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

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

The unfolded protein response in familial amyotrophic lateral sclerosis

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

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

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

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

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

Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)

(2009) Kathryn Volkening et al.   BRAIN RESEARCH

Profiling the Human Protein-DNA Interactome Reveals ERK2 as a Transcriptional Repressor of Interferon Signaling

(2009) Shaohui Hu et al.   CELL

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

(2009) C. Hetz et al.   GENES & DEVELOPMENT

Amyotrophic Lateral Sclerosis-linked Mutant SOD1 Sequesters Hu Antigen R (HuR) and TIA-1-related Protein (TIAR)

(2009) Liang Lu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

(2009) Hristelina Ilieva et al.   JOURNAL OF CELL BIOLOGY

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1

(2008) H. Nishitoh et al.   GENES & DEVELOPMENT