ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

Aberrant association of misfolded SOD1 with Na+/K+ATPase-α3 impairs its activity and contributes to motor neuron vulnerability in ALS

(2015) Céline Ruegsegger et al.   ACTA NEUROPATHOLOGICA

Mitochondrial membrane disruption by aggregation products of ALS-causing superoxide dismutase-1 mutants

(2015) Mohammad Salehi et al.   INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES

Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1

(2015) Adrian Israelson et al.   NEURON

Structural and kinetic analysis of protein-aggregate strains in vivo using binary epitope mapping

(2015) Johan Bergh et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Experimental transmissibility of mutant SOD1 motor neuron disease

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

Non-native Soluble Oligomers of Cu/Zn Superoxide Dismutase (SOD1) Contain a Conformational Epitope Linked to Cytotoxicity in Amyotrophic Lateral Sclerosis (ALS)

(2014) Rachel L. Redler et al.   BIOCHEMISTRY

Identification of a Misfolded Region in Superoxide Dismutase 1 That Is Exposed in Amyotrophic Lateral Sclerosis

(2014) Melissa S. Rotunno et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases

(2014) Jacob I Ayers et al.   Acta Neuropathologica Communications

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1

(2013) S. Pickles et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

Disruption of mitochondrial membrane integrity induced by amyloid aggregates arising from variants of SOD1

(2013) Azarakhsh Oladzad Abbasabadi et al.   INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES

Small Peptides against the Mutant SOD1/Bcl-2 Toxic Mitochondrial Complex Restore Mitochondrial Function and Cell Viability in Mutant SOD1-Mediated ALS

(2013) W. Tan et al.   JOURNAL OF NEUROSCIENCE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Clinical Neurogenetics

(2013) Matthew B. Harms et al.   NEUROLOGIC CLINICS

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

(2013) Smita Saxena et al.   NEURON

PINK1-Phosphorylated Mitofusin 2 Is a Parkin Receptor for Culling Damaged Mitochondria

(2013) Y. Chen et al.   SCIENCE

An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

(2013) Melissa S. Rotunno et al.   Frontiers in Cellular Neuroscience

Misfolded SOD1 and ALS: Zeroing in on mitochondria

(2012) Sarah Pickles et al.   Amyotrophic Lateral Sclerosis

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

(2012) Takao Fujisawa et al.   ANNALS OF NEUROLOGY

Mitochondrial VDAC1: Function in Cell Life and Death and a Target for Cancer Therapy

(2012) V. Shoshan-Barmatz et al.   CURRENT MEDICINAL CHEMISTRY

Targeting of Monomer/Misfolded SOD1 as a Therapeutic Strategy for Amyotrophic Lateral Sclerosis

(2012) H.-N. Liu et al.   JOURNAL OF NEUROSCIENCE

Cellular toxicity of mutant SOD1 protein is linked to an easily soluble, non-aggregated form in vitro

(2012) Terrell E. Brotherton et al.   NEUROBIOLOGY OF DISEASE

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

(2012) T. E. Brotherton et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mechanisms of amyloid fibril formation - focus on domain-swapping

(2011) Eva Žerovnik et al.   FEBS Journal

Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states

(2011) Mercedes Prudencio et al.   Molecular Neurodegeneration

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

(2011) Miranda L. Tradewell et al.   NEUROBIOLOGY OF DISEASE

Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

(2011) Christine Vande Velde 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

Quality Control of Mitochondrial Proteostasis

(2011) M. J. Baker et al.   Cold Spring Harbor Perspectives in Biology

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

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

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

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

(2010) Karin Forsberg et al.   PLoS One

Amyloid fibrils

(2010) Roma N. Rambaran et al.   Prion

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import

(2010) Q. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Emerging roles of mitochondrial proteases in neurodegeneration

(2009) Paola Martinelli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Qualitative determination of superoxide release at both sides of the mitochondrial inner membrane by capillary electrophoretic analysis of the oxidation products of triphenylphosphonium hydroethidine

(2009) Xin Xu et al.   FREE RADICAL BIOLOGY AND MEDICINE

Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

(2008) Hibiki Kawamata et al.   HUMAN MOLECULAR GENETICS

Lysyl-tRNA Synthetase Is a Target for Mutant SOD1 Toxicity in Mitochondria

(2008) Hibiki Kawamata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA