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

Silencing strategies for therapy of SOD1-mediated ALS

(2017) Brigitte van Zundert et al.   NEUROSCIENCE LETTERS

Disease Mechanisms in ALS: Misfolded SOD1 Transferred Through Exosome-Dependent and Exosome-Independent Pathways

(2016) Judith M. Silverman et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Defining SOD1 ALS natural history to guide therapeutic clinical trial design

(2016) Taha Bali et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways

(2015) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

Sporadic and hereditary amyotrophic lateral sclerosis (ALS)

(2015) Senda Ajroud-Driss et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Molecular Chaperones in Cellular Protein Folding: The Birth of a Field

(2014) Arthur L. Horwich   CELL

How the Nucleus Copes with Proteotoxic Stress

(2014) Yoko Shibata et al.   CURRENT BIOLOGY

Dysregulation of the proteasome increases the toxicity of ALS-linked mutant SOD1

(2014) Akira Kitamura et al.   GENES TO CELLS

Delayed Disease Onset and Extended Survival in the SOD1G93A Rat Model of Amyotrophic Lateral Sclerosis after Suppression of Mutant SOD1 in the Motor Cortex

(2014) G. M. Thomsen et al.   JOURNAL OF NEUROSCIENCE

SOD1 Function and Its Implications for Amyotrophic Lateral Sclerosis Pathology

(2014) Rosie K. A. Bunton-Stasyshyn et al.   NEUROSCIENTIST

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

Cellular maintenance of nuclear protein homeostasis

(2013) Pamela S. Gallagher et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Substrate Recognition in Nuclear Protein Quality Control Degradation Is Governed by Exposed Hydrophobicity That Correlates with Aggregation and Insolubility

(2013) Eric K. Fredrickson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Immunodetection of Disease-Associated Conformers of Mutant Cu/Zn Superoxide Dismutase 1 Selectively Expressed in Degenerating Neurons in Amyotrophic Lateral Sclerosis

(2013) Javier Sábado et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

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

(2013) Kengo Homma et al.   MOLECULAR CELL

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol

(2013) Bernadett Kalmar et al.   PHARMACOLOGY & THERAPEUTICS

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

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

Impaired proteasome function in sporadic amyotrophic lateral sclerosis

(2012) Edor Kabashi et al.   Amyotrophic Lateral Sclerosis

Live Cell Imaging of Protein Dislocation from the Endoplasmic Reticulum

(2012) Yongwang Zhong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Sequence and structural analyses of nuclear export signals in the NESdb database

(2012) Darui Xu et al.   MOLECULAR BIOLOGY OF THE CELL

Differential Involvement of Optineurin in Amyotrophic Lateral Sclerosis With or Without SOD1 Mutations

(2011) Han-Xiang Deng   ARCHIVES OF NEUROLOGY

Importin β Interacts with the Endoplasmic Reticulum-associated Degradation Machinery and Promotes Ubiquitination and Degradation of Mutant α1-Antitrypsin

(2011) Yongwang Zhong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Protein localization in disease and therapy

(2011) M.-C. Hung et al.   JOURNAL OF CELL SCIENCE

Disorder Targets Misorder in Nuclear Quality Control Degradation: A Disordered Ubiquitin Ligase Directly Recognizes Its Misfolded Substrates

(2011) Joel C. Rosenbaum et al.   MOLECULAR CELL

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

FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis

(2010) Han-Xiang Deng et al.   ANNALS OF NEUROLOGY

Recognition of nuclear targeting signals by Karyopherin-β proteins

(2010) Darui Xu et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

SOD1 Mutations Targeting Surface Hydrogen Bonds Promote Amyotrophic Lateral Sclerosis without Reducing Apo-state Stability

(2010) Roberth Byström et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

(2010) Francois Gros-Louis et al.   JOURNAL OF NEUROCHEMISTRY

NES consensus redefined by structures of PKI-type and Rev-type nuclear export signals bound to CRM1

(2010) Thomas Güttler et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Nuclear localization sequence of FUS and induction of stress granules by ALS mutants

(2010) Jozsef Gal et al.   NEUROBIOLOGY OF AGING

Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag – Implications for Research into Amyotrophic Lateral Sclerosis (ALS)

(2010) James C. Stevens et al.   PLoS One

Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation

(2009) Zheng Ying et al.   HUMAN MOLECULAR GENETICS

An ALS-Linked Mutant SOD1 Produces a Locomotor Defect Associated with Aggregation and Synaptic Dysfunction When Expressed in Neurons of Caenorhabditis elegans

(2009) Jiou Wang et al.   PLoS Genetics

Nuclear bodies in neurodegenerative disease

(2008) John Woulfe   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Design of Peptide Inhibitors for the Importin α/β Nuclear Import Pathway by Activity-Based Profiling

(2008) Shunichi Kosugi et al.   CHEMISTRY & BIOLOGY