Walking the tightrope: proteostasis and neurodegenerative disease

Oxidative Stress: Emerging Mitochondrial and Cellular Themes and Variations in Neuronal Injury

(2017) Gavin C. Higgins et al.   JOURNAL OF ALZHEIMERS DISEASE

The Biology of Proteostasis in Aging and Disease

(2015) Johnathan Labbadia et al.   Annual Review of Biochemistry

Targeting Hsp90/Hsp70-Based Protein Quality Control for Treatment of Adult Onset Neurodegenerative Diseases

(2015) William B. Pratt et al.   Annual Review of Pharmacology and Toxicology

Widespread Proteome Remodeling and Aggregation in Aging C. elegans

(2015) Dirk M. Walther et al.   CELL

Neuronal Serotonin Release Triggers the Heat Shock Response in C. elegans in the Absence of Temperature Increase

(2015) Marcus C. Tatum et al.   CURRENT BIOLOGY

Compartment-specific aggregases direct distinct nuclear and cytoplasmic aggregate deposition

(2015) S. B. Miller et al.   EMBO JOURNAL

The Chemical Biology of Molecular Chaperones—Implications for Modulation of Proteostasis

(2015) Kristoffer R. Brandvold et al.   JOURNAL OF MOLECULAR BIOLOGY

Spatially Organized Aggregation of Misfolded Proteins as Cellular Stress Defense Strategy

(2015) Stephanie B.M. Miller et al.   JOURNAL OF MOLECULAR BIOLOGY

Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation

(2015) Guilian Xu et al.   JOURNAL OF NEUROCHEMISTRY

Cell type– and brain region–resolved mouse brain proteome

(2015) Kirti Sharma et al.   NATURE NEUROSCIENCE

Cmr1/WDR76 defines a nuclear genotoxic stress body linking genome integrity and protein quality control

(2015) Irene Gallina et al.   Nature Communications

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

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

Preventing α-synuclein aggregation: The role of the small heat-shock molecular chaperone proteins

(2014) Dezerae Cox et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A systems biology investigation of neurodegenerative dementia reveals a pivotal role of autophagy

(2014) Laura Caberlotto et al.   BMC Systems Biology

TRPV1 Pain Receptors Regulate Longevity and Metabolism by Neuropeptide Signaling

(2014) Céline E. Riera et al.   CELL

Celastrol, an oral heat shock activator, ameliorates multiple animal disease models of cell death

(2014) Sudhish Sharma et al.   CELL STRESS & CHAPERONES

Small heat-shock proteins: important players in regulating cellular proteostasis

(2014) Teresa M. Treweek et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

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

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

The deubiquitinase USP28 controls intestinal homeostasis and promotes colorectal cancer

(2014) Markus E. Diefenbacher et al.   JOURNAL OF CLINICAL INVESTIGATION

Frontotemporal dementia and its subtypes: a genome-wide association study

(2014) Raffaele Ferrari et al.   LANCET NEUROLOGY

A three-dimensional human neural cell culture model of Alzheimer’s disease

(2014) Se Hoon Choi et al.   NATURE

Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models

(2014) Sami J Barmada et al.   Nature Chemical Biology

The unfolded-protein-response sensor IRE-1α regulates the function of CD8α+ dendritic cells

(2014) Fabiola Osorio et al.   NATURE IMMUNOLOGY

The amyloid state and its association with protein misfolding diseases

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

Promoting Autophagic Clearance: Viable Therapeutic Targets in Alzheimer’s Disease

(2014) Lauren G. Friedman et al.   Neurotherapeutics

Hypochlorite-induced structural modifications enhance the chaperone activity of human  2-macroglobulin

(2014) A. R. Wyatt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein aggregation can inhibit clathrin-mediated endocytosis by chaperone competition

(2014) A. Yu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

HSF-1-mediated cytoskeletal integrity determines thermotolerance and life span

(2014) N. A. Baird et al.   SCIENCE

PICALM modulates autophagy activity and tau accumulation

(2014) Kevin Moreau et al.   Nature Communications

From cartoon to real time MRI: in vivo monitoring of phagocyte migration in mouse brain

(2014) Yuki Mori et al.   Scientific Reports

A Chaperome Subnetwork Safeguards Proteostasis in Aging and Neurodegenerative Disease

(2014) Marc Brehme et al.   Cell Reports

Extracellular Chaperones and Proteostasis

(2013) Amy R. Wyatt et al.   Annual Review of Biochemistry

Molecular Chaperone Functions in Protein Folding and Proteostasis

(2013) Yujin E. Kim et al.   Annual Review of Biochemistry

Boosting chaperone-mediated autophagy in vivo mitigates α-synuclein-induced neurodegeneration

(2013) Maria Xilouri et al.   BRAIN

Mitochondrial protein quality control in health and disease

(2013) Michael J Baker et al.   BRITISH JOURNAL OF PHARMACOLOGY

XBP-1 Is a Cell-Nonautonomous Regulator of Stress Resistance and Longevity

(2013) Rebecca C. Taylor et al.   CELL

Neuronal Ubiquitin Homeostasis

(2013) Jada Hallengren et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

A Platform to View Huntingtin Exon 1 Aggregation Flux in the Cell Reveals Divergent Influences from Chaperones hsp40 and hsp70

(2013) Angelique R. Ormsby et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Negative Regulation of 26S Proteasome Stability via Calpain-mediated Cleavage of Rpn10 Subunit upon Mitochondrial Dysfunction in Neurons

(2013) Qian Huang 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

Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration

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

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

(2013) Bernadett Kalmar et al.   PHARMACOLOGY & THERAPEUTICS

Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2013) Sergey S. Novoselov et al.   PLoS One

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

Autophagic cell death exists

(2012) Peter G.H. Clarke et al.   Autophagy

Locus coeruleus neurons are resistant to dysfunction and degeneration by maintaining free ubiquitin levels although total ubiquitin levels decrease upon disruption of polyubiquitin gene Ubb

(2012) Chul-Woo Park et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies

(2012) Tina W. Han et al.   CELL

The pathways of mitophagy for quality control and clearance of mitochondria

(2012) G Ashrafi et al.   CELL DEATH AND DIFFERENTIATION

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

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

Avoiding the oligomeric state: αB-crystallin inhibits fragmentation and induces dissociation of apolipoprotein C-II amyloid fibrils

(2012) Katrina J. Binger et al.   FASEB JOURNAL

Transitory phases of autophagic death and programmed necrosis during superoxide-induced neuronal cell death

(2012) Gavin C. Higgins et al.   FREE RADICAL BIOLOGY AND MEDICINE

Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype

(2012) Kate Roberts et al.   GLIA

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function

(2012) Owen A. Brady et al.   HUMAN MOLECULAR GENETICS

Effect ofαB-Crystallin on Protein Aggregation inDrosophila

(2012) Nguyen Trong Tue et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

Induced pluripotent stem cells as tools for disease modelling and drug discovery in Alzheimer’s disease

(2012) Lezanne Ooi et al.   JOURNAL OF NEURAL TRANSMISSION

Over-expression of heat shock factor 1 phenocopies the effect of chronic inhibition of TOR by rapamycin and is sufficient to ameliorate Alzheimer's-like deficits in mice modeling the disease

(2012) Anson Pierce et al.   JOURNAL OF NEUROCHEMISTRY

The interplay of neuronal mitochondrial dynamics and bioenergetics: Implications for Parkinson's disease

(2012) Victor S. Van Laar et al.   NEUROBIOLOGY OF DISEASE

GRP78 counteracts cell death and protein aggregation caused by mutant huntingtin proteins

(2012) Yufeng Jiang et al.   NEUROSCIENCE LETTERS

Withaferin A Induces Proteasome Inhibition, Endoplasmic Reticulum Stress, the Heat Shock Response and Acquisition of Thermotolerance

(2012) Saad Khan et al.   PLoS One

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

Heat Shock Protein–Inducing Compounds as Therapeutics to Restore Proteostasis in Atrial Fibrillation

(2012) Femke Hoogstra-Berends et al.   TRENDS IN CARDIOVASCULAR MEDICINE

Nucleocytoplasmic Coagulation: An Injury-Induced Aggregation Event that Disulfide Crosslinks Proteins and Facilitates Their Removal by Plasmin

(2012) Andre L. Samson et al.   Cell Reports

Regulation of HSF1 Function in the Heat Stress Response: Implications in Aging and Disease

(2011) Julius Anckar et al.   Annual Review of Biochemistry

Neuronal Intrinsic Mechanisms of Axon Regeneration

(2011) Kai Liu et al.   Annual Review of Neuroscience

Ubiquitin editing enzyme UCH L1 and microtubule dynamics: Implication in mitosis

(2011) Anjali Bheda et al.   CELL CYCLE

Autophagic activity in cortical neurons under acute oxidative stress directly contributes to cell death

(2011) Gavin C. Higgins et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Clusterin facilitates in vivo clearance of extracellular misfolded proteins

(2011) Amy R. Wyatt et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Ubiquitin-dependent mitochondrial protein degradation

(2011) Jin-Mi Heo et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

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

Suppression of Alzheimer's Disease-Related Phenotypes by Expression of Heat Shock Protein 70 in Mice

(2011) T. Hoshino et al.   JOURNAL OF NEUROSCIENCE

Generalization of the Prion Hypothesis to Other Neurodegenerative Diseases: An Imperfect Fit

(2011) Will C. Guest et al.   JOURNAL OF TOXICOLOGY AND ENVIRONMENTAL HEALTH-PART A-CURRENT ISSUES

Molecular chaperones in protein folding and proteostasis

(2011) F. Ulrich Hartl et al.   NATURE

Identifying polyglutamine protein species in situ that best predict neurodegeneration

(2011) Jason Miller et al.   Nature Chemical Biology

The role of ubiquitylation in nerve cell development

(2011) Hiroshi Kawabe et al.   NATURE REVIEWS NEUROSCIENCE

Neuronal Autophagy as a Mediator of Life and Death

(2011) Julien Puyal et al.   NEUROSCIENTIST

Transmission of endoplasmic reticulum stress and pro-inflammation from tumor cells to myeloid cells

(2011) N. R. Mahadevan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Neuronal GPCR Controls Innate Immunity by Regulating Noncanonical Unfolded Protein Response Genes

(2011) J. Sun et al.   SCIENCE

Exploring the link between glucocerebrosidase mutations and parkinsonism

(2011) Wendy Westbroek et al.   TRENDS IN MOLECULAR MEDICINE

Ubiquitination in Postsynaptic Function and Plasticity

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

A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases

(2010) Valeria Crippa et al.   Autophagy

Ubiquitin–Proteasome System and mitochondria — Reciprocity

(2010) Nurit Livnat-Levanon et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

The Interaction of αB-Crystallin with Mature α-Synuclein Amyloid Fibrils Inhibits Their Elongation

(2010) Christopher A. Waudby et al.   BIOPHYSICAL JOURNAL

Heat-shock protein 70 modulates toxic extracellular α-synuclein oligomers and rescues trans-synaptic toxicity

(2010) Karin M. Danzer et al.   FASEB JOURNAL

Chemical modulators of autophagy as biological probes and potential therapeutics

(2010) Angeleen Fleming et al.   Nature Chemical Biology

Heat shock factors: integrators of cell stress, development and lifespan

(2010) Malin Åkerfelt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Chaperone networks: Tipping the balance in protein folding diseases

(2010) Cindy Voisine et al.   NEUROBIOLOGY OF DISEASE

Sortilin-Mediated Endocytosis Determines Levels of the Frontotemporal Dementia Protein, Progranulin

(2010) Fenghua Hu et al.   NEURON

Regulation of Mammalian Autophagy in Physiology and Pathophysiology

(2010) Brinda Ravikumar et al.   PHYSIOLOGICAL REVIEWS

The HSP70 Molecular Chaperone Is Not Beneficial in a Mouse Model of α-synucleinopathy

(2010) Derya R. Shimshek et al.   PLoS One

Widespread Protein Aggregation as an Inherent Part of Aging in C. elegans

(2010) Della C. David et al.   PLOS BIOLOGY

Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases

(2009) Gary E. Gibson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A nonfibrin macromolecular cofactor for tPA-mediated plasmin generation following cellular injury

(2009) A. L. Samson et al.   BLOOD

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

(2009) Maria Teresa Giordana et al.   BRAIN PATHOLOGY

An Inhibitor of a Deubiquitinating Enzyme Regulates Ubiquitin Homeostasis

(2009) Yoko Kimura et al.   CELL

Conditioning Protects C. elegans from Lethal Effects of Enteropathogenic E. coli by Activating Genes that Regulate Lifespan and Innate Immunity

(2009) Akwasi Anyanful et al.   Cell Host & Microbe

The Molecular Chaperone Hsp90 Modulates Intermediate Steps of Amyloid Assembly of the Parkinson-related Protein α-Synuclein

(2009) S. Fabio Falsone 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

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

Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging

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

An Analytical Solution to the Kinetics of Breakable Filament Assembly

(2009) T. P. J. Knowles et al.   SCIENCE

Protease Activation of α2-Macroglobulin Modulates a Chaperone-like Action with Broad Specificity

(2008) Katie French et al.   BIOCHEMISTRY

Caveolin-1-dependent apoptosis induced by fibrin degradation products

(2008) Y.-H. Guo et al.   BLOOD

α2-Macroglobulin and Haptoglobin Suppress Amyloid Formation by Interacting with Prefibrillar Protein Species

(2008) Justin J. Yerbury et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Misfolded proteins partition between two distinct quality control compartments

(2008) Daniel Kaganovich et al.   NATURE

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE