SOD1A4Vaggregation alters ubiquitin homeostasis in a cell model of ALS

Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis

(2018) Amit U Joshi et al.   EMBO Molecular Medicine

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

(2017) Prajwal Ciryam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Ubiquitin Code in the Ubiquitin-Proteasome System and Autophagy

(2017) Yong Tae Kwon et al.   TRENDS IN BIOCHEMICAL SCIENCES

Continued 26S proteasome dysfunction in mouse brain cortical neurons impairs autophagy and the Keap1-Nrf2 oxidative defence pathway

(2017) Aslihan Ugun-Klusek et al.   Cell Death & Disease

Insulin and diet-induced changes in the ubiquitin-modified proteome of rat liver

(2017) Shilpa R. Nagarajan et al.   PLoS One

Walking the tightrope: proteostasis and neurodegenerative disease

(2016) Justin J. Yerbury et al.   JOURNAL OF NEUROCHEMISTRY

A transcriptional signature of Alzheimer’s disease is associated with a metastable subproteome at risk for aggregation

(2016) Prajwal Ciryam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Bimolecular complementation affinity purification (BiCAP) reveals dimer-specific protein interactions for ERBB2 dimers

(2016) David R. Croucher et al.   Science Signaling

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

(2016) Kelly L. Williams et al.   Nature Communications

Bimolecular complementation affinity purification (BiCAP) reveals dimer-specific protein interactions for ERBB2 dimers

(2016) David R. Croucher et al.   Science Signaling

Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity

(2016) Luke McAlary et al.   Frontiers in Neuroscience

SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation

(2015) Rafaa Zeineddine et al.   Molecular Neurodegeneration

Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features

(2015) Shu Yang et al.   NEUROTOXICITY RESEARCH

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

Supersaturation is a major driving force for protein aggregation in neurodegenerative diseases

(2015) Prajwal Ciryam et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

(2015) Natalie E. Farrawell et al.   Scientific Reports

Distinct effects of ubiquitin overexpression on NMJ structure and motor performance in mice expressing catalytically inactive USP14

(2015) Jada H. Vaden et al.   Frontiers in Molecular Neuroscience

TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord

(2014) Johannes Brettschneider et al.   ACTA NEUROPATHOLOGICA

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1 

(2014) A. Hyrskyluoto et al.   HUMAN MOLECULAR GENETICS

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

(2014) E. L. Scotter et al.   JOURNAL OF CELL SCIENCE

Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy

(2014) Thomas M. Wishart et al.   JOURNAL OF CLINICAL INVESTIGATION

STRING v10: protein–protein interaction networks, integrated over the tree of life

(2014) Damian Szklarczyk et al.   NUCLEIC ACIDS RESEARCH

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

Mitochondrial impairment triggers cytosolic oxidative stress and cell death following proteasome inhibition

(2014) Sunita Maharjan et al.   Scientific Reports

Extracellular wildtype and mutant SOD1 induces ER–Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells

(2013) Vinod Sundaramoorthy et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis

(2013) Julie D. Atkin et al.   JOURNAL OF NEUROCHEMISTRY

Controversies and priorities in amyotrophic lateral sclerosis

(2013) Martin R Turner et al.   LANCET NEUROLOGY

Genetics of amyotrophic lateral sclerosis: an update

(2013) Sheng Chen et al.   Molecular Neurodegeneration

Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration

(2013) Andrey S Tsvetkov et al.   Nature Chemical Biology

Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis

(2013) Luke McAlary et al.   Scientific Reports

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

Protein Aggregation and Defective RNA Metabolism as Mechanisms for Motor Neuron Damage

(2012) N. Ticozzi et al.   CNS & Neurological Disorders-Drug Targets

Ataxin-3 Regulates Aggresome Formation of Copper-Zinc Superoxide Dismutase (SOD1) by Editing K63-linked Polyubiquitin Chains

(2012) Hongfeng Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis

(2012) Yoshitaka Tashiro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

(2012) Wenjun Song et al.   NEUROBIOLOGY OF DISEASE

Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity

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

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

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

The role of ubiquitylation in nerve cell development

(2011) Hiroshi Kawabe et al.   NATURE REVIEWS NEUROSCIENCE

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

(2011) Christine Vande Velde et al.   PLoS One

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

(2011) Christian Münch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ubiquitination in Postsynaptic Function and Plasticity

(2010) Angela M. Mabb et al.   Annual Review of Cell and Developmental Biology

Ubiquitin/proteasome pathway impairment in neurodegeneration: therapeutic implications

(2010) Qian Huang et al.   APOPTOSIS

Cytoscape 2.8: new features for data integration and network visualization

(2010) M. E. Smoot et al.   BIOINFORMATICS

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

(2010) Lindsey R. Fischer et al.   BRAIN

TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis

(2009) Maria Teresa Giordana et al.   BRAIN PATHOLOGY

Loss of PINK1 Function Promotes Mitophagy through Effects on Oxidative Stress and Mitochondrial Fission

(2009) Ruben K. Dagda et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis†

(2008) Cristina Cheroni et al.   HUMAN MOLECULAR GENETICS

MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification

(2008) Jürgen Cox et al.   NATURE BIOTECHNOLOGY