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Title
Proteostasis disturbance in amyotrophic lateral sclerosis
Authors
Keywords
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Journal
HUMAN MOLECULAR GENETICS
Volume 26, Issue R2, Pages R91-R104
Publisher
Oxford University Press (OUP)
Online
2017-07-12
DOI
10.1093/hmg/ddx274
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Note: Only part of the references are listed.- Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities
- (2017) Fiona M. Menzies et al. NEURON
- The ER proteostasis network in ALS: Determining the differential motoneuron vulnerability
- (2017) Pablo Rozas et al. NEUROSCIENCE LETTERS
- Defective recognition of LC3B by mutant SQSTM1/p62 implicates impairment of autophagy as a pathogenic mechanism in ALS-FTLD
- (2016) Alice Goode et al. Autophagy
- Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)
- (2016) Daniel J Klionsky et al. Autophagy
- The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis
- (2016) Han-Jou Chen et al. BRAIN
- The unfolded protein response: mechanisms and therapy of neurodegeneration
- (2016) Heather L. Smith et al. BRAIN
- Proteostasis impairment in ALS
- (2016) Céline Ruegsegger et al. BRAIN RESEARCH
- C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles
- (2016) Kyung-Ha Lee et al. CELL
- ALS‐linked protein disulfide isomerase variants cause motor dysfunction
- (2016) Ute Woehlbier et al. EMBO JOURNAL
- Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death
- (2016) Chantal Sellier et al. EMBO JOURNAL
- The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy
- (2016) Christopher P Webster et al. EMBO JOURNAL
- Droplet organelles?
- (2016) Edward M Courchaine et al. EMBO JOURNAL
- The integrated stress response
- (2016) Karolina Pakos‐Zebrucka et al. EMBO REPORTS
- Heat shock proteins as potential targets for protective strategies in neurodegeneration
- (2016) Harm H Kampinga et al. LANCET NEUROLOGY
- Decoding ALS: from genes to mechanism
- (2016) J. Paul Taylor et al. NATURE
- The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease
- (2016) Aaron R. Haeusler et al. NATURE REVIEWS NEUROSCIENCE
- Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis
- (2016) Sebum Lee et al. NEURON
- Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110
- (2016) Maria Nagy et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS–FTD-linkedUBQLN2mutations
- (2016) Nhat T. T. Le et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Demen
- (2016) Ruxandra Dafinca et al. STEM CELLS
- Principles and Properties of Stress Granules
- (2016) David S.W. Protter et al. TRENDS IN CELL BIOLOGY
- Common Molecular Pathways in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
- (2016) Jochen H. Weishaupt et al. TRENDS IN MOLECULAR MEDICINE
- CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia
- (2016) Kelly L. Williams et al. Nature Communications
- Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase
- (2016) Eiichi Tokuda et al. Acta Neuropathologica Communications
- The unfolded protein response in neurodegenerative diseases: a neuropathological perspective
- (2015) Wiep Scheper et al. ACTA NEUROPATHOLOGICA
- The Role of Endoplasmic Reticulum Stress in Human Pathology
- (2015) Scott A. Oakes et al. Annual Review of Pathology-Mechanisms of Disease
- Role of autophagy in the pathogenesis of amyotrophic lateral sclerosis
- (2015) Jae Keun Lee et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients
- (2015) Paloma Gonzalez-Perez et al. GENE
- Emerging mechanisms of molecular pathology in ALS
- (2015) Owen M. Peters et al. JOURNAL OF CLINICAL INVESTIGATION
- 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
- Chaperones in Neurodegeneration
- (2015) I. Lindberg et al. JOURNAL OF NEUROSCIENCE
- Proteostasis control by the unfolded protein response
- (2015) Claudio Hetz et al. NATURE CELL BIOLOGY
- Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia
- (2015) Axel Freischmidt et al. NATURE NEUROSCIENCE
- Guanabenz Treatment Accelerates Disease in a Mutant SOD1 Mouse Model of ALS
- (2015) Fernando G. Vieira et al. PLoS One
- Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS
- (2015) Shuying Sun et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- An ALS disease mutation in Cdc48/p97 impairs 20S proteasome binding and proteolytic communication
- (2015) Dominik Barthelme et al. PROTEIN SCIENCE
- Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit
- (2015) I. Das et al. SCIENCE
- Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways
- (2015) E. T. Cirulli et al. SCIENCE
- In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11
- (2015) Rita-Eva Varga et al. PLoS Genetics
- Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress
- (2014) Yong-Jie Zhang et al. ACTA NEUROPATHOLOGICA
- Altered macroautophagy in the spinal cord of SOD1 mutant mice
- (2014) Liang Li et al. Autophagy
- Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis
- (2014) Melissa Nassif et al. Autophagy
- MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis
- (2014) Xiaojie Zhang et al. Autophagy
- Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events
- (2014) Meredith E. Jackrel et al. CELL
- Common Ground: Stem Cell Approaches Find Shared Pathways Underlying ALS
- (2014) Soledad Matus et al. Cell Stem Cell
- Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1
- (2014) Evangelos Kiskinis et al. Cell Stem Cell
- Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis
- (2014) Mayuko Osaka et al. HUMAN MOLECULAR GENETICS
- The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis
- (2014) H. Meyer et al. JOURNAL OF CELL SCIENCE
- 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
- Spastic paraplegia proteins spastizin and spatacsin mediate autophagic lysosome reformation
- (2014) Jaerak Chang et al. JOURNAL OF CLINICAL INVESTIGATION
- Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models
- (2014) Sami J Barmada et al. Nature Chemical Biology
- Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases
- (2014) Claudio Hetz et al. NATURE REVIEWS NEUROSCIENCE
- Autophagy meets fused in sarcoma-positive stress granules
- (2014) Soledad Matus et al. NEUROBIOLOGY OF AGING
- Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis
- (2014) Lijun Wang et al. NEUROBIOLOGY OF DISEASE
- Guanabenz delays the onset of disease symptoms, extends lifespan, improves motor performance and attenuates motor neuron loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis
- (2014) H.-Q. Jiang et al. NEUROSCIENCE
- Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation
- (2014) Yvette C. Wong et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Getting RIDD of RNA: IRE1 in cell fate regulation
- (2014) M. Maurel et al. TRENDS IN BIOCHEMICAL SCIENCES
- Targeting autophagy in neurodegenerative diseases
- (2014) René L. Vidal et al. TRENDS IN PHARMACOLOGICAL SCIENCES
- Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology
- (2013) Elisa Teyssou et al. ACTA NEUROPATHOLOGICA
- Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons
- (2013) Karen Castillo et al. Autophagy
- When ER stress reaches a dead end
- (2013) Hery Urra et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
- Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function
- (2013) J. Ross Buchan et al. CELL
- An enhanced integrated stress response ameliorates mutant SOD1-induced ALS
- (2013) L. Wang et al. HUMAN MOLECULAR GENETICS
- Altered localization, abnormal modification and loss of function of Sigma receptor-1 in amyotrophic lateral sclerosis
- (2013) J. Prause et al. HUMAN MOLECULAR GENETICS
- Controversies and priorities in amyotrophic lateral sclerosis
- (2013) Martin R Turner et al. LANCET NEUROLOGY
- 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
- Rapamycin increases survival in ALS mice lacking mature lymphocytes
- (2013) Kim A Staats et al. Molecular Neurodegeneration
- The epidemiology of ALS: a conspiracy of genes, environment and time
- (2013) Ammar Al-Chalabi et al. Nature Reviews Neurology
- Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival
- (2013) Smita Saxena et al. NEURON
- The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol
- (2013) Bernadett Kalmar et al. PHARMACOLOGY & THERAPEUTICS
- Functional Contribution of the Transcription Factor ATF4 to the Pathogenesis of Amyotrophic Lateral Sclerosis
- (2013) Soledad Matus et al. PLoS One
- ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation
- (2013) Adam K. Walker et al. PLoS One
- Measurement of autophagy flux in the nervous system in vivo
- (2013) K Castillo et al. Cell Death & Disease
- Impaired proteasome function in sporadic amyotrophic lateral sclerosis
- (2012) Edor Kabashi et al. Amyotrophic Lateral Sclerosis
- mTOR Signaling in Growth Control and Disease
- (2012) Mathieu Laplante et al. CELL
- Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis
- (2012) Yoshitaka Tashiro et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS
- (2012) N. Bernard-Marissal et al. JOURNAL OF NEUROSCIENCE
- Autophagy modulation as a potential therapeutic target for diverse diseases
- (2012) David C. Rubinsztein et al. NATURE REVIEWS DRUG DISCOVERY
- Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase
- (2012) Manal A. Farg et al. NEUROBIOLOGY OF AGING
- 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
- Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43
- (2012) I.-F. Wang et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis
- (2011) Amr Al-Saif et al. ANNALS OF NEUROLOGY
- SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
- (2011) Faisal Fecto ARCHIVES OF NEUROLOGY
- Rapamycin treatment augments motor neuron degeneration in SOD1G93Amouse model of amyotrophic lateral sclerosis
- (2011) Xiaojie Zhang et al. Autophagy
- Autophagy in Spinal Cord Motor Neurons in Sporadic Amyotrophic Lateral Sclerosis
- (2011) Shoichi Sasaki JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
- (2011) Han-Xiang Deng et al. NATURE
- Heat shock transcription factor 1 as a therapeutic target in neurodegenerative diseases
- (2011) Daniel W. Neef et al. NATURE REVIEWS DRUG DISCOVERY
- Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
- (2011) Laura Ferraiuolo et al. Nature Reviews Neurology
- Dietary restriction but not rapamycin extends disease onset and survival of the H46R/H48Q mouse model of ALS
- (2011) Arunabh Bhattacharya et al. NEUROBIOLOGY OF AGING
- Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
- (2011) Mariely DeJesus-Hernandez et al. NEURON
- A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
- (2011) Alan E. Renton et al. NEURON
- Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells
- (2011) Giovanni Nardo et al. PLoS One
- Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: Implication for protein aggregation and immune response
- (2011) Caterina Bendotti et al. PROGRESS IN NEUROBIOLOGY
- The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation
- (2011) P. Walter et al. SCIENCE
- VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD
- (2010) Emilie Tresse et al. Autophagy
- SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis
- (2010) A. Orlacchio et al. BRAIN
- The unfolded protein response in familial amyotrophic lateral sclerosis
- (2010) L. Wang et al. HUMAN MOLECULAR GENETICS
- Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy
- (2010) C. Rothenberg et al. HUMAN MOLECULAR GENETICS
- Rapamycin Protects against Neuron Death in In Vitro andIn Vivo Models of Parkinson's Disease
- (2010) C. Malagelada et al. JOURNAL OF NEUROSCIENCE
- Mutations of optineurin in amyotrophic lateral sclerosis
- (2010) Hirofumi Maruyama et al. NATURE
- Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
- (2010) Daryl A Bosco et al. NATURE NEUROSCIENCE
- Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
- (2010) Janel O. Johnson et al. NEURON
- Loss of ALS2/Alsin Exacerbates Motor Dysfunction in a SOD1H46R-Expressing Mouse ALS Model by Disturbing Endolysosomal Trafficking
- (2010) Shinji Hadano et al. PLoS One
- Inhibition of mTOR by Rapamycin Abolishes Cognitive Deficits and Reduces Amyloid-β Levels in a Mouse Model of Alzheimer's Disease
- (2010) Patricia Spilman et al. PLoS One
- Novel Antibodies Reveal Inclusions Containing Non-Native SOD1 in Sporadic ALS Patients
- (2010) Karin Forsberg et al. PLoS One
- Rethinking ALS: The FUS about TDP-43
- (2009) Clotilde Lagier-Tourenne et al. CELL
- XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
- (2009) C. Hetz et al. GENES & DEVELOPMENT
- Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease
- (2009) Jeong-Sun Ju et al. JOURNAL OF CELL BIOLOGY
- Fine-Tuning of the Unfolded Protein Response: Assembling the IRE1α Interactome
- (2009) Claudio Hetz et al. MOLECULAR CELL
- A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice
- (2009) Smita Saxena et al. NATURE NEUROSCIENCE
- 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
- ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
- (2008) H. Nishitoh et al. GENES & DEVELOPMENT
- 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
- VAPB interacts with and modulates the activity of ATF6
- (2008) C. Gkogkas et al. HUMAN MOLECULAR GENETICS
- Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease
- (2008) B. Ravikumar et al. JOURNAL OF CELL SCIENCE
- Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS
- (2008) Bernadett Kalmar et al. JOURNAL OF NEUROCHEMISTRY
- ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB
- (2008) Hiroaki Suzuki et al. JOURNAL OF NEUROCHEMISTRY
- TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis
- (2008) Vivianna M Van Deerlin et al. LANCET NEUROLOGY
- TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
- (2008) Edor Kabashi et al. NATURE GENETICS
- Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
- (2008) Julie D. Atkin et al. NEUROBIOLOGY OF DISEASE
- Neuron-specific overexpression of the co-chaperone Bcl-2-associated athanogene-1 in superoxide dismutase 1G93A–transgenic mice
- (2008) G. Rohde et al. NEUROSCIENCE
- Lithium delays progression of amyotrophic lateral sclerosis
- (2008) F. Fornai et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- 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
- Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases
- (2007) Jiou Wang et al. NEUROBIOLOGY OF AGING
- Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression
- (2007) Paul S. Sharp et al. NEUROBIOLOGY OF DISEASE
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