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

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

(2012) Takao Fujisawa et al.   ANNALS OF NEUROLOGY

Hsp70 targets Hsp100 chaperones to substrates for protein disaggregation and prion fragmentation

(2012) Juliane Winkler et al.   JOURNAL OF CELL BIOLOGY

RPN-6 determines C. elegans longevity under proteotoxic stress conditions

(2012) David Vilchez et al.   NATURE

An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

(2012) S. Guareschi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

A cellular perspective on conformational disease: the role of genetic background and proteostasis networks

(2010) Tali Gidalevitz et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Mutation-dependent Polymorphism of Cu,Zn-Superoxide Dismutase Aggregates in the Familial Form of Amyotrophic Lateral Sclerosis

(2010) Yoshiaki Furukawa 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

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

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

Converging concepts of protein folding in vitro and in vivo

(2009) F Ulrich Hartl et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

Destabilizing Protein Polymorphisms in the Genetic Background Direct Phenotypic Expression of Mutant SOD1 Toxicity

(2009) Tali Gidalevitz et al.   PLoS Genetics

Protein Quality Control: On IPODs and Other JUNQ

(2008) Katrin Bagola et al.   CURRENT BIOLOGY

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

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

A Limited Role for Disulfide Cross-linking in the Aggregation of Mutant SOD1 Linked to Familial Amyotrophic Lateral Sclerosis

(2008) Celeste M. Karch 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

Misfolded proteins partition between two distinct quality control compartments

(2008) Daniel Kaganovich et al.   NATURE

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