Misfolded SOD1 and ALS: Zeroing in on mitochondria

Metabolic and functional differences between brain and spinal cord mitochondria underlie different predisposition to pathology

(2011) Alexander V. Panov et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

ALS-Causing SOD1 Mutations Promote Production of Copper-Deficient Misfolded Species

(2011) Philbert Ip et al.   JOURNAL OF MOLECULAR BIOLOGY

Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients

(2011) Per Zetterström et al.   JOURNAL OF NEUROCHEMISTRY

In Vivo Pathogenic Role of Mutant SOD1 Localized in the Mitochondrial Intermembrane Space

(2011) A. Igoudjil et al.   JOURNAL OF NEUROSCIENCE

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

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

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

Neurotoxic Species of Misfolded SOD1G93ARecognized by Antibodies Against the P2X4Subunit of the ATP Receptor Accumulate in Damaged Neurons of Transgenic Animal Models of Amyotrophic Lateral Sclerosis

(2010) Sara Hernández et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

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

Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis

(2009) Hsueh-Ning Liu et al.   ANNALS OF NEUROLOGY

Axonal Mitochondrial Clusters Containing Mutant SOD1 in Transgenic Models of ALS

(2009) Jose R. Sotelo-Silveira et al.   ANTIOXIDANTS & REDOX SIGNALING

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

(2009) Mercedes Prudencio et al.   HUMAN MOLECULAR GENETICS

Voltage-dependent Anion Channel 1-based Peptides Interact with Bcl-2 to Prevent Antiapoptotic Activity

(2009) Nir Arbel 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

Mitochondrial Ubiquitin Ligase MITOL Ubiquitinates Mutant SOD1 and Attenuates Mutant SOD1-induced Reactive Oxygen Species Generation

(2009) Ryo Yonashiro et al.   MOLECULAR BIOLOGY OF THE CELL

Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS

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

Deleterious Role of Superoxide Dismutase in the Mitochondrial Intermembrane Space

(2008) Gundars Goldsteins et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Denaturational Stress Induces Formation of Zinc-Deficient Monomers of Cu,Zn Superoxide Dismutase: Implications for Pathogenesis in Amyotrophic Lateral Sclerosis

(2008) Vikram Khipple Mulligan et al.   JOURNAL OF MOLECULAR BIOLOGY

Unfolding and Folding Kinetics of Amyotrophic Lateral Sclerosis-Associated Mutant Cu,Zn Superoxide Dismutases

(2008) Jessica A.O. Rumfeldt et al.   JOURNAL OF MOLECULAR BIOLOGY

Mutant SOD1 from spinal cord of G93A rats is destabilized and binds to inner mitochondrial membrane

(2008) Toni Ahtoniemi et al.   NEUROBIOLOGY OF DISEASE

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