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

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

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

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

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

A protein homeostasis signature in healthy brains recapitulates tissue vulnerability to Alzheimers disease

(2016) R. Freer et al.   Science Advances

Structural basis for mutation-induced destabilization of profilin 1 in ALS

(2015) Sivakumar Boopathy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Differential Scales of Protein Quality Control

(2014) Suzanne Wolff et al.   CELL

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

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

SQSTM1 mutations – Bridging Paget disease of bone and ALS/FTLD

(2014) Sarah L. Rea et al.   EXPERIMENTAL CELL RESEARCH

Misfolded Polyglutamine, Polyalanine, and Superoxide Dismutase 1 Aggregate via Distinct Pathways in the Cell

(2014) Saskia Polling et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Profilin 1 Associates with Stress Granules and ALS-Linked Mutations Alter Stress Granule Dynamics

(2014) M. D. Figley et al.   JOURNAL OF NEUROSCIENCE

A draft map of the human proteome

(2014) Min-Sik Kim et al.   NATURE

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

The amyloid state and its association with protein misfolding diseases

(2014) Tuomas P. J. Knowles et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The BioGRID interaction database: 2015 update

(2014) Andrew Chatr-aryamontri 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

Controversies and priorities in amyotrophic lateral sclerosis

(2013) Martin R Turner et al.   LANCET NEUROLOGY

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism

(2012) Brian A. Keller et al.   ACTA NEUROPATHOLOGICA

Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity

(2012) Alice Brockington et al.   ACTA NEUROPATHOLOGICA

The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro

(2012) Justin J. Yerbury et al.   CELL STRESS & CHAPERONES

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

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

PaxDb, a Database of Protein Abundance Averages Across All Three Domains of Life

(2012) M. Wang et al.   MOLECULAR & CELLULAR PROTEOMICS

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology

(2011) Yasuko Toyoshima et al.   ACTA NEUROPATHOLOGICA

TDP-43 pathological changes in early onset familial and sporadic Alzheimer’s disease, late onset Alzheimer’s disease and Down’s Syndrome: association with age, hippocampal sclerosis and clinical phenotype

(2011) Yvonne S. Davidson et al.   ACTA NEUROPATHOLOGICA

Phosphatase and tensin homologue/protein kinase B pathway linked to motor neuron survival in human superoxide dismutase 1-related amyotrophic lateral sclerosis

(2011) Janine Kirby et al.   BRAIN

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

ANS Binding Reveals Common Features of Cytotoxic Amyloid Species

(2010) Benedetta Bolognesi et al.   ACS Chemical Biology

Interaction Between ABCB1 and Professional Exposure to Organochlorine Insecticides in Parkinson Disease

(2010) Fabien Dutheil et al.   ARCHIVES OF NEUROLOGY

Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice

(2010) Daniel Bergemalm et al.   JOURNAL OF NEUROCHEMISTRY

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado–Joseph disease

(2009) Chun-Feng Tan et al.   ACTA NEUROPATHOLOGICA

Deleterious Variants of FIG4, a Phosphoinositide Phosphatase, in Patients with ALS

(2009) Clement Y. Chow et al.   AMERICAN JOURNAL OF HUMAN GENETICS

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

(2009) Brian S. Johnson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterization of Detergent-Insoluble Proteins in ALS Indicates a Causal Link between Nitrative Stress and Aggregation in Pathogenesis

(2009) Manuela Basso et al.   PLoS One

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

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

(2009) Tali Gidalevitz et al.   PLoS Genetics

Proteomic identification of novel proteins associated with Lewy bodies

(2008) Qiangwei Xia   Frontiers in Bioscience-Landmark

Detergent-insoluble Aggregates Associated with Amyotrophic Lateral Sclerosis in Transgenic Mice Contain Primarily Full-length, Unmodified Superoxide Dismutase-1

(2008) Bryan F. Shaw et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prediction of Aggregation-Prone Regions in Structured Proteins

(2008) Gian Gaetano Tartaglia et al.   JOURNAL OF MOLECULAR BIOLOGY

TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia

(2008) C C Weihl et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Colocalization of Transactivation-Responsive DNA-Binding Protein 43 and Huntingtin in Inclusions of Huntington Disease

(2008) Claudia Schwab et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Misfolded proteins partition between two distinct quality control compartments

(2008) Daniel Kaganovich et al.   NATURE

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE