Structural Properties and Interaction Partners of Familial ALS-Associated SOD1 Mutants

The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS

(2019) Yulia Dzhashiashvili et al.   NEUROBIOLOGY OF DISEASE

Macrophage migration inhibitory factor: A multifaceted cytokine implicated in multiple neurological diseases

(2018) Marcel F. Leyton-Jaimes et al.   EXPERIMENTAL NEUROLOGY

The role of tRNA synthetases in neurological and neuromuscular disorders

(2018) Veronika Boczonadi et al.   FEBS LETTERS

SOD1 in Amyotrophic Lateral Sclerosis: “Ambivalent” Behavior Connected to the Disease

(2018) Orietta Pansarasa et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications

(2018) Ruth Chia et al.   LANCET NEUROLOGY

Autophagy as a common pathway in amyotrophic lateral sclerosis

(2018) Dao K.H. Nguyen et al.   NEUROSCIENCE LETTERS

Large SOD1 aggregates, unlike trimeric SOD1, do not impact cell viability in a model of amyotrophic lateral sclerosis

(2018) Cheng Zhu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Effects of maturation on the conformational free-energy landscape of SOD1

(2018) Robert M. Culik et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Atomic structures of corkscrew-forming segments of SOD1 reveal varied oligomer conformations

(2018) Smriti Sangwan et al.   PROTEIN SCIENCE

MIF inhibits the formation and toxicity of misfolded SOD1 amyloid aggregates: implications for familial ALS

(2018) Neta Shvil et al.   Cell Death & Disease

A small-molecule inhibitor of SOD1-Derlin-1 interaction ameliorates pathology in an ALS mouse model

(2018) Naomi Tsuburaya et al.   Nature Communications

Abnormal RNA stability in amyotrophic lateral sclerosis

(2018) E. M. Tank et al.   Nature Communications

VDAC1 as Pharmacological Target in Cancer and Neurodegeneration: Focus on Its Role in Apoptosis

(2018) Andrea Magrì et al.   Frontiers in Chemistry

Investigation of Anti-SOD1 Antibodies Yields New Structural Insight into SOD1 Misfolding and Surprising Behavior of the Antibodies Themselves

(2018) Ryan S. Atlasi et al.   ACS Chemical Biology

Parkin is a disease modifier in the mutant SOD1 mouse model of ALS

(2018) Gloria M Palomo et al.   EMBO Molecular Medicine

Impaired proteostasis during skeletal muscle aging

(2018) Raquel Fernando et al.   FREE RADICAL BIOLOGY AND MEDICINE

The Cost of Long Catalytic Loops in Folding and Stability of the ALS-Associated Protein SOD1

(2018) Fan Yang et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

G3BP1 promotes DNA binding and activation of cGAS

(2018) Zhao-Shan Liu et al.   NATURE IMMUNOLOGY

Amyotrophic lateral sclerosis-related mutant superoxide dismutase 1 aggregates inhibit 14-3-3-mediated cell survival by sequestration into the JUNQ compartment

(2017) Ju-Hwang Park et al.   HUMAN MOLECULAR GENETICS

Sex specific activation of the ERα axis of the mitochondrial UPR (UPRmt) in the G93A-SOD1 mouse model of familial ALS

(2017) Amanjot K Riar et al.   HUMAN MOLECULAR GENETICS

Copper-zinc superoxide dismutase is activated through a sulfenic acid intermediate at a copper ion entry site

(2017) Morgan M. Fetherolf et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Unraveling new functions of superoxide dismutase using yeast model system: Beyond its conventional role in superoxide radical scavenging

(2017) Woo-Hyun Chung   JOURNAL OF MICROBIOLOGY

Amyotrophic lateral sclerosis

(2017) Michael A van Es et al.   LANCET

Acylation of Superoxide Dismutase 1 (SOD1) at K122 Governs SOD1-Mediated Inhibition of Mitochondrial Respiration

(2017) Courtney J. Banks et al.   MOLECULAR AND CELLULAR BIOLOGY

Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?

(2017) Kurt J. De Vos et al.   NEUROBIOLOGY OF DISEASE

Genetics of amyotrophic lateral sclerosis

(2017) P. Corcia et al.   REVUE NEUROLOGIQUE

Nuclear export of misfolded SOD1 mediated by a normally buried NES-like sequence reduces proteotoxicity in the nucleus

(2017) Yongwang Zhong et al.   eLife

Pathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates

(2017) Fernando Bartolome et al.   Scientific Reports

ALS mutant SOD1 interacts with G3BP1 and affects stress granule dynamics

(2016) Jozsef Gal et al.   ACTA NEUROPATHOLOGICA

Mitochondria‐associated membrane collapse is a common pathomechanism in SIGMAR1 ‐ and SOD1 ‐linked ALS

(2016) Seiji Watanabe et al.   EMBO Molecular Medicine

Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS

(2016) Marcel F. Leyton-Jaimes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

(2016) Sarah Pickles et al.   Acta Neuropathologica Communications

Mechanism for transforming cytosolic SOD1 into integral membrane proteins of organelles by ALS-causing mutations

(2015) Liangzhong Lim et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Calcium binding to gatekeeper residues flanking aggregation-prone segments underlies non-fibrillar amyloid traits in superoxide dismutase 1 (SOD1)

(2015) Sílvia G. Estácio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Neurofilament dynamics and involvement in neurological disorders

(2015) Benoit J. Gentil et al.   CELL AND TISSUE RESEARCH

Neurofilament assembly and function during neuronal development

(2015) Adi Laser-Azogui et al.   CURRENT OPINION IN CELL BIOLOGY

Conformational Disorder of the Most Immature Cu, Zn-Superoxide Dismutase Leading to Amyotrophic Lateral Sclerosis

(2015) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Parkin Protects Against Misfolded SOD1 Toxicity by Promoting Its Aggresome Formation and Autophagic Clearance

(2015) Cheryl Yung et al.   MOLECULAR NEUROBIOLOGY

A systematic immunoprecipitation approach reinforces the concept of common conformational alterations in amyotrophic lateral sclerosis-linked SOD1 mutants

(2015) Takao Fujisawa et al.   NEUROBIOLOGY OF DISEASE

Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis

(2015) Elizabeth A. Proctor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis

(2015) Francesco Tafuri et al.   Frontiers in Cellular Neuroscience

Pre-emptive Quality Control Protects the ER from Protein Overload via the Proximity of ERAD Components and SRP

(2015) Hisae Kadowaki et al.   Cell Reports

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

Dysfunction of Endocytic Kinase AAK1 in ALS

(2014) Bingxing Shi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Insights into the Role of the Unusual Disulfide Bond in Copper-Zinc Superoxide Dismutase

(2014) Kevin Sea et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

The phenotypic variability of amyotrophic lateral sclerosis

(2014) Bart Swinnen et al.   Nature Reviews Neurology

Amyotrophic Lateral Sclerosis Genetic Studies

(2014) Ji He et al.   NEUROSCIENTIST

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

(2014) Chi Kwan Tsang et al.   Nature Communications

Roles of Vascular Endothelial Growth Factor in Amyotrophic Lateral Sclerosis

(2014) Ana Catarina Pronto-Laborinho et al.   Biomed Research International

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

(2014) Nico P. Dantuma et al.   Frontiers in Molecular Neuroscience

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

(2013) Rachele A. Saccon et al.   BRAIN

Characterization of human sporadic ALS biomarkers in the familial ALS transgenic mSOD1G93A mouse model

(2013) Eitan Lilo et al.   HUMAN MOLECULAR GENETICS

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1

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

HDAC6 Regulates Mutant SOD1 Aggregation through Two SMIR Motifs and Tubulin Acetylation

(2013) Jozsef Gal 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

Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis

(2013) Pei-Yi Lin et al.   Molecular Neurodegeneration

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities

(2013) Eiichi Tokuda et al.   NEUROBIOLOGY OF DISEASE

Global structural motions from the strain of a single hydrogen bond

(2013) J. Danielsson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Aggregation-triggering segments of SOD1 fibril formation support a common pathway for familial and sporadic ALS

(2013) M. I. Ivanova 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

Nuclear Localization of Human SOD1 and Mutant SOD1-Specific Disruption of Survival Motor Neuron Protein Complex in Transgenic Amyotrophic Lateral Sclerosis Mice

(2012) Barry Gertz et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model of ALS

(2012) Valentina Cappello et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

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

The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis

(2012) Hirotaka Tanaka et al.   Scientific Reports

Autophagy: Renovation of Cells and Tissues

(2011) Noboru Mizushima et al.   CELL

HuR function in disease

(2011) Subramanya Srikantan   Frontiers in Bioscience-Landmark

Regulation of Autophagy by Neuropathological Protein TDP-43

(2011) Jayarama Krishnan Bose et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Proteins That Bind to Misfolded Mutant Superoxide Dismutase-1 in Spinal Cords from Transgenic Amyotrophic Lateral Sclerosis (ALS) Model Mice

(2011) Per Zetterström et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

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

(2011) Mercedes Prudencio et al.   Molecular Neurodegeneration

Colocalization of 14-3-3 Proteins with SOD1 in Lewy Body-Like Hyaline Inclusions in Familial Amyotrophic Lateral Sclerosis Cases and the Animal Model

(2011) Yoko Okamoto et al.   PLoS One

CHIP promotes the degradation of mutant SOD1 by reducing its interaction with VCP and S6/S6′ subunits of 26S proteasome

(2010) Jin-Sun Choi et al.   Animal Cells and Systems

p62/SQSTM1 is required for Parkin-induced mitochondrial clustering but not mitophagy; VDAC1 is dispensable for both

(2010) Derek Narendra et al.   Autophagy

RNF43 interacts with NEDL1 and regulates p53-mediated transcription

(2010) Keisuke Shinada et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport

(2010) Ping Shi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2

(2010) Steve Pedrini et al.   HUMAN MOLECULAR GENETICS

Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis

(2010) Nichole A. Reyes et al.   JOURNAL OF CLINICAL INVESTIGATION

Disulfide-Reduced ALS Variants of Cu, Zn Superoxide Dismutase Exhibit Increased Populations of Unfolded Species

(2010) Can Kayatekin et al.   JOURNAL OF MOLECULAR BIOLOGY

Folding Catalysis by Transient Coordination of Zn2+to the Cu Ligands of the ALS-Associated Enzyme Cu/Zn Superoxide Dismutase 1

(2010) Lina Leinartaitė et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

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

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase

(2010) Shinji Higashi et al.   NEUROCHEMISTRY INTERNATIONAL

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

(2010) Adrian Israelson et al.   NEURON

Familial amyotrophic lateral sclerosis-linked mutant SOD1 aberrantly interacts with tubulin

(2009) Tomohiro Kabuta et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Structural and Biophysical Properties of the Pathogenic SOD1 Variant H46R/H48Q†

(2009) Duane D. Winkler et al.   BIOCHEMISTRY

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

Vascular endothelial growth factor prevents G93A-SOD1-induced motor neuron degeneration

(2009) J. Simon Lunn et al.   Developmental Neurobiology

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

Sequestosome 1/p62 links familial ALS mutant SOD1 to LC3 via an ubiquitin-independent mechanism

(2009) Jozsef Gal et al.   JOURNAL OF NEUROCHEMISTRY

Functional features cause misfolding of the ALS-provoking enzyme SOD1

(2009) A. Nordlund et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS

(2009) J. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutants

(2009) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

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

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

Mutant copper-zinc superoxide dismutase associated with amyotrophic lateral sclerosis binds to adenine/uridine-rich stability elements in the vascular endothelial growth factor 3′-untranslated region

(2008) Xuelin Li et al.   JOURNAL OF NEUROCHEMISTRY

Autophagy fights disease through cellular self-digestion

(2008) Noboru Mizushima et al.   NATURE

Adherent Monomer-Misfolded SOD1

(2008) Yasuhiro Watanabe et al.   PLoS One