Proteostasis disturbance in amyotrophic lateral sclerosis

2016, Decoding ALS: from genes to mechanism, Nature, 539, 197, 10.1038/nature20413

2013, Controversies and priorities in amyotrophic lateral sclerosis, Lancet. Neurol, 12, 310, 10.1016/S1474-4422(13)70036-X

2013, The epidemiology of ALS: a conspiracy of genes, environment and time, Nat. Rev. Neurol, 9, 617, 10.1038/nrneurol.2013.203

2011, Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis, Nat. Rev. Neurol, 7, 616, 10.1038/nrneurol.2011.152

2011, Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS, Neuron, 72, 245, 10.1016/j.neuron.2011.09.011

2011, A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD, Neuron, 72, 257, 10.1016/j.neuron.2011.09.010

2003, Unfolding the role of protein misfolding in neurodegenerative diseases, Nat. Rev. Neurosci, 4, 49, 10.1038/nrn1007

2005, The genetic epidemiology of neurodegenerative disease, J. Clin. Invest, 115, 1449, 10.1172/JCI24761

2010, Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS, Nat. Neurosci, 13, 1396, 10.1038/nn.2660

2010, Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients, PLoS One, 5, e11552., 10.1371/journal.pone.0011552

2006, Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis, Science, 314, 130, 10.1126/science.1134108

2008, Adapting proteostasis for disease intervention, Science, 319, 916, 10.1126/science.1141448

2014, Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases, Nat. Rev. Neurosci, 15, 233, 10.1038/nrn3689

2016, The unfolded protein response: mechanisms and therapy of neurodegeneration, Brain, 139, 2113, 10.1093/brain/aww101

2015, The unfolded protein response in neurodegenerative diseases: a neuropathological perspective, Acta Neuropathol, 130, 315, 10.1007/s00401-015-1462-8

2015, Proteostasis control by the unfolded protein response, 1

2016, Proteostasis impairment in ALS, Brain Res, 1648, 571, 10.1016/j.brainres.2016.03.032

2017, The ER proteostasis network in ALS: Determining the differential motoneuron vulnerability, Neurosci. Lett, 636, 9, 10.1016/j.neulet.2016.04.066

2014, Targeting autophagy in neurodegenerative diseases, Trends Pharmacol. Sci, 35, 583, 10.1016/j.tips.2014.09.002

2017, Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities, Neuron, 93, 1015, 10.1016/j.neuron.2017.01.022

2002, The Ubiquitin-Proteasome Proteolytic Pathway: Destruction for the Sake of Construction, Physiol. Rev, 82, 373, 10.1152/physrev.00027.2001

2012, Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: implication for protein aggregation and immune response, Prog. Neurobiol, 97, 101, 10.1016/j.pneurobio.2011.10.001

2012, Impaired proteasome function in sporadic amyotrophic lateral sclerosis, Amyotroph. Lateral Scler, 13, 367, 10.3109/17482968.2012.686511

2002, Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity, J. Biol. Chem, 277, 36793, 10.1074/jbc.M206559200

2002, Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis, J. Neurochem, 83, 1030, 10.1046/j.1471-4159.2002.01211.x

2004, Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis, J. Neurochem, 89, 1325, 10.1111/j.1471-4159.2004.02453.x

2005, Accumulation of human SOD1 and ubiquitinated deposits in the spinal cord of SOD1G93A mice during motor neuron disease progression correlates with a decrease of proteasome, Neurobiol. Dis, 18, 509, 10.1016/j.nbd.2004.12.007

2009, Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis, Hum. Mol. Genet, 18, 82, 10.1093/hmg/ddn319

2015, Emerging mechanisms of molecular pathology in ALS, J. Clin. Invest, 125, 2548., 10.1172/JCI82693

2014, Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress, Acta Neuropathol, 128, 505, 10.1007/s00401-014-1336-5

2014, The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis, J. Cell Sci, 127, 3877, 10.1242/jcs.093831

2010, Exome sequencing reveals VCP mutations as a cause of familial ALS, Neuron, 68, 857, 10.1016/j.neuron.2010.11.036

2015, An ALS disease mutation in Cdc48/p97 impairs 20S proteasome binding and proteolytic communication, Protein Sci, 24, 1521, 10.1002/pro.2740

2004, Ubiquilin interacts with ubiquitylated proteins and proteasome through its ubiquitin-associated and ubiquitin-like domains, FEBS Lett, 566, 110, 10.1016/j.febslet.2004.04.031

2011, Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia, Nature, 477, 211, 10.1038/nature10353

2016, Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations, Proc. Natl. Acad. Sci. U. S. A, 113, E7580, 10.1073/pnas.1608432113

2014, Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species, J. Cell Sci, 127, 1263, 10.1242/jcs.140087

2012, Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis, J. Biol. Chem, 287, 42984, 10.1074/jbc.M112.417600

2016, CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia, Nat. Commun, 7, 11253., 10.1038/ncomms11253

2017, Molecular definitions of autophagy and related processes, embo J, 36, 1811, 10.15252/embj.201796697

2016, Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition), Autophagy, 12, 1, 10.1080/15548627.2015.1100356

2006, Loss of autophagy in the central nervous system causes neurodegeneration in mice, Nature, 441, 880, 10.1038/nature04723

2006, Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice, Nature, 441, 885, 10.1038/nature04724

2006, Degradation of amyotrophic lateral sclerosis-linked mutant Cu,Zn-superoxide dismutase proteins by macroautophagy and the proteasome, J. Biol. Chem, 281, 30524, 10.1074/jbc.M603337200

2014, Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models, Nat. Chem. Biol, 10, 677, 10.1038/nchembio.1563

2015, Role of autophagy in the pathogenesis of amyotrophic lateral sclerosis, Biochim. Biophys. Acta, 1852, 2517, 10.1016/j.bbadis.2015.08.005

2012, Autophagy modulation as a potential therapeutic target for diverse diseases, Nat. Rev. Drug Discov, 11, 709, 10.1038/nrd3802

2005, Bcl-2 antiapoptotic proteins inhibit Beclin 1-dependent autophagy, Cell, 122, 927, 10.1016/j.cell.2005.07.002

2000, LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing, embo J, 19, 5720, 10.1093/emboj/19.21.5720

2005, p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death, J. Cell Biol, 171, 603, 10.1083/jcb.200507002

2009, XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy, Genes Dev, 23, 2294, 10.1101/gad.1830709

2011, Autophagy in spinal cord motor neurons in sporadic amyotrophic lateral sclerosis, J. Neuropathol. Exp. Neurol, 70, 349, 10.1097/NEN.0b013e3182160690

2007, Increased autophagy in transgenic mice with a G93A mutant SOD1 gene, Brain Res, 1167, 112, 10.1016/j.brainres.2007.06.045

2008, Altered macroautophagy in the spinal cord of SOD1 mutant mice, Autophagy, 4, 290, 10.4161/auto.5524

2011, Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis, Autophagy, 7, 412, 10.4161/auto.7.4.14541

2004, Alsin is a Rab5 and Rac1 guanine nucleotide exchange factor, J. Biol. Chem, 279, 24612, 10.1074/jbc.M313504200

2008, Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease, J. Cell Sci, 121, 1649, 10.1242/jcs.025726

2001, The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis, Nat. Genet, 29, 160, 10.1038/ng1001-160

2001, A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2, Nat. Genet, 29, 166, 10.1038/ng1001-166

2010, Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking, PLoS One, 5, e9805., 10.1371/journal.pone.0009805

2016, The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease, Nat. Rev. Neurosci, 17, 383, 10.1038/nrn.2016.38

2016, The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy, embo J, 35, 1656, 10.15252/embj.201694401

2016, Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death, embo J, 35, 1276, 10.15252/embj.201593350

2016, 1

2014, Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis, Autophagy, 10, 1256, 10.4161/auto.28784

2016, Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase, Acta Neuropathol. Commun, 4, 6., 10.1186/s40478-016-0274-y

2013, Rapamycin increases survival in ALS mice lacking mature lymphocytes, Mol. Neurodegener, 8, 31., 10.1186/1750-1326-8-31

2012, Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43, Proc. Natl. Acad. Sci, 109, 15024, 10.1073/pnas.1206362109

2013, Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons, Autophagy, 9, 1308, 10.4161/auto.25188

2014, MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis, Autophagy, 10, 588, 10.4161/auto.27710

2012, mTOR signaling in growth control and disease, Cell, 149, 274, 10.1016/j.cell.2012.03.017

2004, Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease, Nat. Genet, 36, 585, 10.1038/ng1362

2006, Rapamycin alleviates toxicity of different aggregate-prone proteins, Hum. Mol. Genet, 15, 433, 10.1093/hmg/ddi458

2010, Rapamycin protects against neuron death in in vitro and in vivo models of Parkinson’s disease, J. Neurosci, 30, 1166, 10.1523/JNEUROSCI.3944-09.2010

2010, Inhibition of mTOR by rapamycin abolishes cognitive deficits and reduces amyloid-beta levels in a mouse model of Alzheimer’s disease, PLoS One, 5, e9979., 10.1371/journal.pone.0009979

2012, Dietary restriction but not rapamycin extends disease onset and survival of the H46R/H48Q mouse model of ALS, Neurobiol. Aging, 33, 1829, 10.1016/j.neurobiolaging.2011.06.002

2013, Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival, Neuron, 80, 80, 10.1016/j.neuron.2013.07.027

2013, Measurement of autophagy flux in the nervous system in vivo, Cell Death Dis, 4, e917., 10.1038/cddis.2013.421

2007, Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein, J. Biol. Chem, 282, 5641, 10.1074/jbc.M609532200

2008, Lithium delays progression of amyotrophic lateral sclerosis, Proc. Natl. Acad. Sci. U. S. A, 105, 2052, 10.1073/pnas.0708022105

2011, SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis, Arch. Neurol, 68, 1440, 10.1001/archneurol.2011.250

2013, Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology, Acta Neuropathol, 125, 511, 10.1007/s00401-013-1090-0

2010, Mutations of optineurin in amyotrophic lateral sclerosis, Nature, 465, 223, 10.1038/nature08971

2016, Defective recognition of LC3B by mutant SQSTM1/p62 implicates impairment of autophagy as a pathogenic mechanism in ALS-FTLD, Autophagy, 12, 1094, 10.1080/15548627.2016.1170257

2014, Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation, Proc. Natl. Acad. Sci. U. S. A, 111, E4439, 10.1073/pnas.1405752111

2010, Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy, Hum. Mol. Genet, 19, 3219, 10.1093/hmg/ddq231

2015, Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis, Hum. Mol. Genet, 24, 1617, 10.1093/hmg/ddu575

2015, Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia, Nat. Neurosci, 18, 631, 10.1038/nn.4000

2015, Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways, Science, 347, 1436, 10.1126/science.aaa3650

2009, Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease, J. Cell Biol, 187, 875, 10.1083/jcb.200908115

2010, VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD, Autophagy, 6, 217, 10.4161/auto.6.2.11014

2013, Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function, Cell, 153, 1461, 10.1016/j.cell.2013.05.037

2014, Spastic paraplegia proteins spastizin and spatacsin mediate autophagic lysosome reformation, J. Clin. Invest, 124, 5249, 10.1172/JCI77598

2010, SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis, Brain, 133, 591, 10.1093/brain/awp325

2015, In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11, PLoS Genet, 11, e1005454., 10.1371/journal.pgen.1005454

2016, Principles and Properties of Stress Granules, Trends Cell Biol, 26, 668, 10.1016/j.tcb.2016.05.004

2008, TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis, Science, 319, 1668, 10.1126/science.1154584

2008, TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis, Nat. Genet, 40, 572, 10.1038/ng.132

2008, TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis, Lancet. Neurol, 7, 409, 10.1016/S1474-4422(08)70071-1

2009, Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis, Science, 323, 1205, 10.1126/science.1166066

2009, Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6, Science, 323, 1208, 10.1126/science.1165942

2009, Rethinking ALS: The FUS about TDP-43, Cell, 136, 1001, 10.1016/j.cell.2009.03.006

2016, Common molecular pathways in amyotrophic lateral sclerosis and frontotemporal dementia, Trends Mol. Med, 22, 769, 10.1016/j.molmed.2016.07.005

2016, Droplet organelles?, embo J, 35, 1603, 10.15252/embj.201593517

2015, Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS? Front, Cell. Neurosci, 9, 423.

2016, C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles, Cell, 167, 774, 10.1016/j.cell.2016.10.002

2014, Autophagy meets fused in sarcoma-positive stress granules, Neurobiol. Aging, 35, 2832, 10.1016/j.neurobiolaging.2014.08.019

2016, In vivo aspects of protein folding and quality control, Science, 353, aac4354-aac4354.

2016, Heat shock proteins as potential targets for protective strategies in neurodegeneration, Lancet. Neurol, 15, 748, 10.1016/S1474-4422(16)00099-5

2001, Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis, J. Biol. Chem, 276, 12791, 10.1074/jbc.M010759200

2001, Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues, Neurobiol. Dis, 8, 933, 10.1006/nbdi.2001.0443

2008, Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases, Neurobiol. Aging, 29, 586, 10.1016/j.neurobiolaging.2006.11.009

2016, The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis, Brain, 139, 1417, 10.1093/brain/aww028

1999, Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis, J. Neurochem, 72, 693, 10.1046/j.1471-4159.1999.0720693.x

2004, CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70, J. Neurochem, 90, 231, 10.1111/j.1471-4159.2004.02486.x

2007, Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS, J. Neurochem, 102, 1497, 10.1111/j.1471-4159.2007.04534.x

2005, Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis, J. Neurochem, 93, 875, 10.1111/j.1471-4159.2005.03054.x

2008, Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression, Neurobiol. Dis, 30, 42, 10.1016/j.nbd.2007.12.002

2008, Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrophic lateral sclerosis, J. Neurochem, 106, 2170

2008, Neuron-specific overexpression of the co-chaperone Bcl-2-associated athanogene-1 in superoxide dismutase 1(G93A)-transgenic mice, Neuroscience, 157, 844, 10.1016/j.neuroscience.2008.09.055

2015, 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, J. Neurochem, 133, 452, 10.1111/jnc.13022

2016, Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110, Proc. Natl. Acad. Sci. U. S. A, 113, 5424, 10.1073/pnas.1604885113

2012, Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity, Proc. Natl. Acad. Sci. U. S. A, 109, 15811, 10.1073/pnas.1205829109

2014, Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events, Cell, 156, 170, 10.1016/j.cell.2013.11.047

2015, Chaperones in neurodegeneration, J. Neurosci, 35, 13853, 10.1523/JNEUROSCI.2600-15.2015

2013, 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, Mol. Neurodegener, 8, 43., 10.1186/1750-1326-8-43

2011, Heat shock transcription factor 1 as a therapeutic target in neurodegenerative diseases, Nat. Rev. Drug Discov, 10, 930, 10.1038/nrd3453

2004, Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice, Nat. Med, 10, 402, 10.1038/nm1021

2008, Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1 mouse model of ALS, J. Neurochem, 107, 339, 10.1111/j.1471-4159.2008.05595.x

2014, The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol, Pharmacol. Ther, 141, 40, 10.1016/j.pharmthera.2013.08.003

2006, Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1, J. Biol. Chem, 281, 30152, 10.1074/jbc.M603393200

2008, Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis, Neurobiol. Dis, 30, 400, 10.1016/j.nbd.2008.02.009

2007, Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis, Brain, 130, 3111, 10.1093/brain/awm190

2014, Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1, Cell Stem Cell, 14, 781, 10.1016/j.stem.2014.03.004

2016, 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, Stem Cells, 34, 2063, 10.1002/stem.2388

2014, Common ground: stem cell approaches find shared pathways underlying ALS, Cell Stem Cell, 14, 697, 10.1016/j.stem.2014.05.001

2009, A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice, Nat. Neurosci, 12, 627, 10.1038/nn.2297

2015, Nat. Neurosci, 227

2012, The unfolded protein response: controlling cell fate decisions under ER stress and beyond, Nat. Rev. Mol. Cell Biol, 13, 89, 10.1038/nrm3270

2011, The unfolded protein response: from stress pathway to homeostatic regulation, Science, 334, 1081, 10.1126/science.1209038

2015, The role of endoplasmic reticulum stress in human pathology, Annu. Rev. Pathol, 10, 173, 10.1146/annurev-pathol-012513-104649

2013, When ER stress reaches a dead end, Biochim. Biophys. Acta, 1833, 3507, 10.1016/j.bbamcr.2013.07.024

2000, ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs, Mol. Cell, 6, 1355, 10.1016/S1097-2765(00)00133-7

2003, An integrated stress response regulates amino acid metabolism and resistance to oxidative stress, Mol. Cell, 11, 619, 10.1016/S1097-2765(03)00105-9

2000, Regulated translation initiation controls stress-induced gene expression in mammalian cells, Mol. Cell, 6, 1099, 10.1016/S1097-2765(00)00108-8

2017, Drug repurposing to target proteostasis and prevent neurodegeneration: accelerating translational efforts, Brain, 140, 1544, 10.1093/brain/awx107

2016, The integrated stress response, EMBO Rep, 17, 1374, 10.15252/embr.201642195

2015, Proteostasis control by the unfolded protein response, Nat. Cell Biol, 17, 829, 10.1038/ncb3184

2014, Getting RIDD of RNA: IRE1 in cell fate regulation, Trends Biochem. Sci, 39, 245, 10.1016/j.tibs.2014.02.008

2009, Fine-tuning of the unfolded protein response: Assembling the IRE1alpha interactome, Mol. Cell, 35, 551, 10.1016/j.molcel.2009.08.021

2006, Autocrine tumor necrosis factor alpha links endoplasmic reticulum stress to the membrane death receptor pathway through IRE1-mediated NF- B activation and down-regulation of TRAF2 expression, Mol. Cell. Biol, 26, 3071, 10.1128/MCB.26.8.3071-3084.2006

2000, Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1, Science, 287, 664, 10.1126/science.287.5453.664

2008, ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1, Genes Dev, 22, 1451, 10.1101/gad.1640108

2016, Activation of HIPK2 promotes ER stress-mediated neurodegeneration in amyotrophic lateral sclerosis, Neuron, 91, 41, 10.1016/j.neuron.2016.05.021

2013, Functional contribution of the transcription factor ATF4 to the pathogenesis of amyotrophic lateral sclerosis, PLoS One, 8, e66672., 10.1371/journal.pone.0066672

2011, The unfolded protein response in familial amyotrophic lateral sclerosis, Hum. Mol. Genet, 20, 1008, 10.1093/hmg/ddq546

2015, Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit, Science, 348, 239, 10.1126/science.aaa4484

2014, 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, Neuroscience, 277, 132, 10.1016/j.neuroscience.2014.03.047

2014, Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis, Neurobiol. Dis, 71, 317, 10.1016/j.nbd.2014.08.010

2014, An enhanced integrated stress response ameliorates mutant SOD1-induced ALS, Hum. Mol. Genet, 23, 2629, 10.1093/hmg/ddt658

2008, VAPB interacts with and modulates the activity of ATF6, Hum. Mol. Genet, 17, 1517, 10.1093/hmg/ddn040

2006, Characterization of amyotrophic lateral sclerosis-linked P56S mutation of vesicle-associated membrane protein-associated protein B (VAPB/ALS8), J. Biol. Chem, 281, 30223, 10.1074/jbc.M605049200

2004, A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis, Am. J. Hum. Genet, 75, 822, 10.1086/425287

2009, 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, J. Neurochem, 108, 973, 10.1111/j.1471-4159.2008.05857.x

2015, Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS, Proc. Natl. Acad. Sci. U. S. A, 112, E6993, 10.1073/pnas.1520639112

2007, Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survival, Cell, 131, 596, 10.1016/j.cell.2007.08.036

2011, A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis, Ann. Neurol, 70, 913, 10.1002/ana.22534

2013, Altered localization, abnormal modification and loss of function of Sigma receptor-1 in amyotrophic lateral sclerosis, Hum. Mol. Genet, 22, 1581, 10.1093/hmg/ddt008

2015, Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients, Gene, 566, 158, 10.1016/j.gene.2015.04.035

2016, ALS-linked protein disulfide isomerase variants cause motor dysfunction, embo J, 35, 845, 10.15252/embj.201592224

2011, Amyotrophic lateral sclerosis multiprotein biomarkers in peripheral blood mononuclear cells, PLoS One, 6, e25545., 10.1371/journal.pone.0025545

2013, ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation, PLoS One, 8, e81170., 10.1371/journal.pone.0081170

2012, Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase, Neurobiol. Aging, 33, 2855, 10.1016/j.neurobiolaging.2012.02.009

2012, Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS, J. Neurosci, 32, 4901, 10.1523/JNEUROSCI.5431-11.2012

2015, Guanabenz Treatment Accelerates Disease in a Mutant SOD1 Mouse Model of ALS, PLoS One, 10, e0135570, 10.1371/journal.pone.0135570