Published in Acta Neuropathol on July 26, 2015
PERK inhibition prevents tau-mediated neurodegeneration in a mouse model of frontotemporal dementia. Acta Neuropathol (2015) 0.97
Mitofusin-mediated ER stress triggers neurodegeneration in pink1/parkin models of Parkinson's disease. Cell Death Dis (2016) 0.87
Mitochondrial dysfunction induces dendritic loss via eIF2α phosphorylation. J Cell Biol (2017) 0.80
Roles of tau protein in health and disease. Acta Neuropathol (2017) 0.80
The entangled ER-mitochondrial axis as a potential therapeutic strategy in neurodegeneration: A tangled duo unchained. Cell Calcium (2016) 0.78
Copper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 Mutations. Int J Mol Sci (2016) 0.77
Endoplasmic reticulum proteostasis impairment in aging. Aging Cell (2017) 0.76
ER stress and the unfolded protein response in neurodegeneration. Nat Rev Neurol (2017) 0.75
NetDecoder: a network biology platform that decodes context-specific biological networks and gene activities. Nucleic Acids Res (2016) 0.75
Commentary: Parkinson disease-linked GBA mutation effects reversed by molecular chaperones in human cell and fly models. Front Neurosci (2016) 0.75
Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly. Cell Death Dis (2017) 0.75
New insights into the unfolded protein response in stem cells. Oncotarget (2016) 0.75
Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology. Acta Neuropathol Commun (2016) 0.75
Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy. Acta Neuropathol (2016) 0.75
Targeting New Candidate Genes by Small Molecules Approaching Neurodegenerative Diseases. Int J Mol Sci (2015) 0.75
Protective Effect of Enalapril against Methionine-Enriched Diet-Induced Hypertension: Role of Endoplasmic Reticulum and Oxidative Stress. Biomed Res Int (2015) 0.75
Thiamine Deficiency and Neurodegeneration: the Interplay Among Oxidative Stress, Endoplasmic Reticulum Stress, and Autophagy. Mol Neurobiol (2016) 0.75
Cellular Regulation of Amyloid Formation in Aging and Disease. Front Neurosci (2017) 0.75
Involvement of Endoplasmic Reticulum Stress in TULP1 Induced Retinal Degeneration. PLoS One (2016) 0.75
PKR involvement in Alzheimer's disease. Alzheimers Res Ther (2017) 0.75
Activation of TGF-β signaling induces cell death via the unfolded protein response in Fuchs endothelial corneal dystrophy. Sci Rep (2017) 0.75
Bidirectional interplay of HSF1 degradation and UPR activation promotes tau hyperphosphorylation. PLoS Genet (2017) 0.75
Omega-3 fatty acids increase the unfolded protein response and improve amyloid-β phagocytosis by macrophages of patients with mild cognitive impairment. FASEB J (2017) 0.75
Fine tuning PERK signaling for neuroprotection. J Neurochem (2017) 0.75
The Endoplasmic Reticulum Unfolded Protein Response in Neurodegenerative Disorders and Its Potential Therapeutic Significance. Front Mol Neurosci (2017) 0.75
Interplay of Energetics and ER Stress Exacerbates Alzheimer's Amyloid-β (Aβ) Toxicity in Yeast. Front Mol Neurosci (2017) 0.75
Inhibition of the integrated stress response reverses cognitive deficits after traumatic brain injury. Proc Natl Acad Sci U S A (2017) 0.75
Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol (1991) 49.03
Staging of brain pathology related to sporadic Parkinson's disease. Neurobiol Aging (2002) 22.40
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron (2011) 20.15
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron (2011) 18.73
XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor. Cell (2001) 18.68
Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase. Nature (1999) 16.97
IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA. Nature (2002) 16.52
Perk is essential for translational regulation and cell survival during the unfolded protein response. Mol Cell (2000) 11.09
Transcriptional induction of genes encoding endoplasmic reticulum resident proteins requires a transmembrane protein kinase. Cell (1993) 9.84
Mammalian transcription factor ATF6 is synthesized as a transmembrane protein and activated by proteolysis in response to endoplasmic reticulum stress. Mol Biol Cell (1999) 9.05
Autophagy is activated for cell survival after endoplasmic reticulum stress. Mol Cell Biol (2006) 8.89
Diabetes mellitus and exocrine pancreatic dysfunction in perk-/- mice reveals a role for translational control in secretory cell survival. Mol Cell (2001) 8.60
A novel mechanism for regulating activity of a transcription factor that controls the unfolded protein response. Cell (1996) 8.17
A selective inhibitor of eIF2alpha dephosphorylation protects cells from ER stress. Science (2005) 7.98
The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins. Nature (1988) 7.98
Alpha-synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models. Science (2006) 7.72
Plasma cell differentiation and the unfolded protein response intersect at the transcription factor XBP-1. Nat Immunol (2003) 7.06
Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci (2004) 6.99
A transmembrane protein with a cdc2+/CDC28-related kinase activity is required for signaling from the ER to the nucleus. Cell (1993) 6.82
Autophagy counterbalances endoplasmic reticulum expansion during the unfolded protein response. PLoS Biol (2006) 6.47
IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response. Genes Dev (2002) 6.37
The transmembrane kinase Ire1p is a site-specific endonuclease that initiates mRNA splicing in the unfolded protein response. Cell (1997) 6.26
Cloning of mammalian Ire1 reveals diversity in the ER stress responses. EMBO J (1998) 5.96
A stress response pathway from the endoplasmic reticulum to the nucleus requires a novel bifunctional protein kinase/endoribonuclease (Ire1p) in mammalian cells. Genes Dev (1998) 5.88
Toxic proteins in neurodegenerative disease. Science (2002) 5.82
Identification and characterization of pancreatic eukaryotic initiation factor 2 alpha-subunit kinase, PEK, involved in translational control. Mol Cell Biol (1998) 5.66
Oligomerization and phosphorylation of the Ire1p kinase during intracellular signaling from the endoplasmic reticulum to the nucleus. EMBO J (1996) 5.46
Transcriptional induction of mammalian ER quality control proteins is mediated by single or combined action of ATF6alpha and XBP1. Dev Cell (2007) 5.45
The PERK eukaryotic initiation factor 2 alpha kinase is required for the development of the skeletal system, postnatal growth, and the function and viability of the pancreas. Mol Cell Biol (2002) 5.36
IRE1alpha kinase activation modes control alternate endoribonuclease outputs to determine divergent cell fates. Cell (2009) 4.65
Oral treatment targeting the unfolded protein response prevents neurodegeneration and clinical disease in prion-infected mice. Sci Transl Med (2013) 4.62
EIF2AK3, encoding translation initiation factor 2-alpha kinase 3, is mutated in patients with Wolcott-Rallison syndrome. Nat Genet (2000) 4.48
ATF6alpha optimizes long-term endoplasmic reticulum function to protect cells from chronic stress. Dev Cell (2007) 4.39
An essential role in liver development for transcription factor XBP-1. Genes Dev (2000) 4.17
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat Neurosci (2009) 3.89
Differential effects of endoplasmic reticulum stress-induced autophagy on cell survival. J Biol Chem (2006) 3.74
The unfolded protein response: a pathway that links insulin demand with beta-cell failure and diabetes. Endocr Rev (2008) 3.56
Sustained translational repression by eIF2α-P mediates prion neurodegeneration. Nature (2012) 3.54
A 22 bp cis-acting element is necessary and sufficient for the induction of the yeast KAR2 (BiP) gene by unfolded proteins. EMBO J (1992) 3.48
Selective inhibition of a regulatory subunit of protein phosphatase 1 restores proteostasis. Science (2011) 3.29
The unfolded protein response sensor IRE1alpha is required at 2 distinct steps in B cell lymphopoiesis. J Clin Invest (2005) 3.16
Expression of A53T mutant but not wild-type alpha-synuclein in PC12 cells induces alterations of the ubiquitin-dependent degradation system, loss of dopamine release, and autophagic cell death. J Neurosci (2001) 3.10
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. Genes Dev (2009) 3.03
Phosphorylation of the translation initiation factor eIF2alpha increases BACE1 levels and promotes amyloidogenesis. Neuron (2008) 2.99
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms. Nature (2004) 2.98
Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet (2011) 2.97
Endoplasmic reticulum stress and the unfolded protein response in cellular models of Parkinson's disease. J Neurosci (2002) 2.83
Identification and rescue of α-synuclein toxicity in Parkinson patient-derived neurons. Science (2013) 2.61
Fine-tuning of the unfolded protein response: Assembling the IRE1alpha interactome. Mol Cell (2009) 2.47
The molecular basis for selective inhibition of unconventional mRNA splicing by an IRE1-binding small molecule. Proc Natl Acad Sci U S A (2012) 2.45
Regulation of basal cellular physiology by the homeostatic unfolded protein response. J Cell Biol (2010) 2.40
The eIF2α kinases: their structures and functions. Cell Mol Life Sci (2013) 2.37
The unfolded protein response pathway in Saccharomyces cerevisiae. Oligomerization and trans-phosphorylation of Ire1p (Ern1p) are required for kinase activation. J Biol Chem (1996) 2.36
Suppression of eIF2α kinases alleviates Alzheimer's disease-related plasticity and memory deficits. Nat Neurosci (2013) 2.35
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. EMBO J (2003) 2.34
Modeling Alzheimer's disease with iPSCs reveals stress phenotypes associated with intracellular Aβ and differential drug responsiveness. Cell Stem Cell (2013) 2.32
ER-phagy: selective autophagy of the endoplasmic reticulum. Autophagy (2007) 2.21
Enhanced bacterial clearance and sepsis resistance in caspase-12-deficient mice. Nature (2006) 2.17
Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response. Nat Cell Biol (1999) 2.14
A role for presenilin-1 in nuclear accumulation of Ire1 fragments and induction of the mammalian unfolded protein response. Cell (1999) 2.11
Stress protein systems of mammalian cells. Am J Physiol (1986) 2.11
The unfolded protein response is activated in Alzheimer's disease. Acta Neuropathol (2005) 2.05
Tau accumulation activates the unfolded protein response by impairing endoplasmic reticulum-associated degradation. J Neurosci (2013) 2.04
Activation of the unfolded protein response in Parkinson's disease. Biochem Biophys Res Commun (2007) 1.98
The endoplasmic reticulum stress-responsive protein GRP78 protects neurons against excitotoxicity and apoptosis: suppression of oxidative stress and stabilization of calcium homeostasis. Exp Neurol (1999) 1.95
Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol Dis (2008) 1.94
Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons. Cell Rep (2014) 1.93
The unfolded protein response is activated in pretangle neurons in Alzheimer's disease hippocampus. Am J Pathol (2009) 1.92
Endoplasmic reticulum stress activates autophagy but not the proteasome in neuronal cells: implications for Alzheimer's disease. Cell Death Differ (2011) 1.86
Pharmacological brake-release of mRNA translation enhances cognitive memory. Elife (2013) 1.85
Endoplasmic reticulum dysfunction in neurological disease. Lancet Neurol (2013) 1.83
Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science (2015) 1.80
The unfolded protein response is associated with early tau pathology in the hippocampus of tauopathies. J Pathol (2012) 1.76
Quality control: ER-associated degradation: protein quality control and beyond. J Cell Biol (2014) 1.75
Induction of liver steatosis and lipid droplet formation in ATF6alpha-knockout mice burdened with pharmacological endoplasmic reticulum stress. Mol Biol Cell (2010) 1.70
Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell (2014) 1.65
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol (2014) 1.54
Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis. Brain (2007) 1.52
Endoplasmic reticulum stress features are prominent in Alzheimer disease but not in prion diseases in vivo. J Neuropathol Exp Neurol (2006) 1.49
Impaired feedback regulation of XBP1 as a genetic risk factor for bipolar disorder. Nat Genet (2003) 1.46
Divergent allosteric control of the IRE1α endoribonuclease using kinase inhibitors. Nat Chem Biol (2012) 1.46
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy. Hum Mol Genet (2012) 1.36
Expression of heat shock proteins in Alzheimer's disease. Neurology (1991) 1.35
Stress responses. Mutations in a translation initiation factor identify the target of a memory-enhancing compound. Science (2015) 1.34
Granulovacuolar degeneration in the ageing brain and in dementia. J Neuropathol Exp Neurol (1977) 1.33
Allosteric inhibition of the IRE1α RNase preserves cell viability and function during endoplasmic reticulum stress. Cell (2014) 1.23
Abeta 1-42 induces mild endoplasmic reticulum stress in an aggregation state-dependent manner. Antioxid Redox Signal (2007) 1.23
The small molecule ISRIB reverses the effects of eIF2α phosphorylation on translation and stress granule assembly. Elife (2015) 1.19
Reexamination of granulovacuolar degeneration. Acta Neuropathol (1991) 1.18
Oligomer-specific Abeta toxicity in cell models is mediated by selective uptake. Biochim Biophys Acta (2008) 1.14
Involvement of double-stranded RNA-dependent protein kinase and phosphorylation of eukaryotic initiation factor-2alpha in neuronal degeneration. J Neurochem (2002) 1.11
Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease. J Biol Chem (2009) 1.10
Druggable sensors of the unfolded protein response. Nat Chem Biol (2014) 1.09
Genetic variation of promoter sequence modulates XBP1 expression and genetic risk for vitiligo. PLoS Genet (2009) 1.07
Increased ER stress during motor neuron degeneration in a transgenic mouse model of amyotrophic lateral sclerosis. Neurol Res (2007) 1.07
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo. Neurobiol Dis (2013) 1.07
IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux. Hum Mol Genet (2011) 1.03
Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity. Cell Death Dis (2015) 1.02
The unfolded protein response is associated with early tau pathology in the hippocampus of tauopathies. J Pathol (2012) 1.76
Neuroinflammation - an early event in both the history and pathogenesis of Alzheimer's disease. Neurodegener Dis (2010) 1.66
Amyloid Beta induces oxidative stress-mediated blood-brain barrier changes in capillary amyloid angiopathy. Antioxid Redox Signal (2011) 1.29
Abeta 1-42 induces mild endoplasmic reticulum stress in an aggregation state-dependent manner. Antioxid Redox Signal (2007) 1.23
Endoplasmic reticulum protein quality control in neurodegenerative disease: the good, the bad and the therapy. Curr Med Chem (2009) 1.14
Microglia activation in sepsis: a case-control study. J Neuroinflammation (2007) 1.13
DNA polymerase-beta is expressed early in neurons of Alzheimer's disease brain and is loaded into DNA replication forks in neurons challenged with beta-amyloid. J Neurosci (2006) 0.99
Transglutaminases and transglutaminase-catalyzed cross-links colocalize with the pathological lesions in Alzheimer's disease brain. Brain Pathol (2008) 0.96
The unfolded protein response and proteostasis in Alzheimer disease: preferential activation of autophagy by endoplasmic reticulum stress. Autophagy (2011) 0.95
Stages of granulovacuolar degeneration: their relation to Alzheimer's disease and chronic stress response. Acta Neuropathol (2011) 0.93
Antioxidative defense mechanisms controlled by Nrf2: state-of-the-art and clinical perspectives in neurodegenerative diseases. Arch Toxicol (2014) 0.92
Cholinergic imbalance in the multiple sclerosis hippocampus. Acta Neuropathol (2011) 0.91
Presence of tissue transglutaminase in granular endoplasmic reticulum is characteristic of melanized neurons in Parkinson's disease brain. Brain Pathol (2010) 0.90
Interleukin-1 beta-induced expression of the prostaglandin E-receptor subtype EP3 in U373 astrocytoma cells depends on protein kinase C and nuclear factor-kappaB. J Neurochem (2006) 0.90
Neuroinflammation and blood-brain barrier changes in capillary amyloid angiopathy. Neurodegener Dis (2012) 0.87
Amyloid beta peptide (25-35) activates protein kinase C leading to cyclooxygenase-2 induction and prostaglandin E2 release in primary midbrain astrocytes. Neurochem Int (2006) 0.86
Neuroinflammation in plaque and vascular beta-amyloid disorders: clinical and therapeutic implications. Neurodegener Dis (2008) 0.85
Novel role of transglutaminase 1 in corpora amylacea formation? Neurobiol Aging (2009) 0.85
The nature of the cell cycle in neurons: focus on a "non-canonical" pathway of DNA replication causally related to death. Biochim Biophys Acta (2006) 0.85
Intracellular accumulation of aggregated pyroglutamate amyloid beta: convergence of aging and Aβ pathology at the lysosome. Age (Dordr) (2012) 0.84
Innate immunity and the etiology of late-onset Alzheimer's disease. Neurodegener Dis (2012) 0.82
Unfolded protein response activates glycogen synthase kinase-3 via selective lysosomal degradation. Neurobiol Aging (2013) 0.82
Adult human microglia secrete cytokines when exposed to neurotoxic prion protein peptide: no intermediary role for prostaglandin E2. Brain Res (2002) 0.82
Increased expression of the oligopeptidase THOP1 is a neuroprotective response to Abeta toxicity. Neurobiol Dis (2008) 0.82
Quantification of amyloid-beta 40 in cerebrospinal fluid. J Immunol Methods (2009) 0.81
Ubiquilin 2 is not associated with tau pathology. PLoS One (2013) 0.80
Physiological and pathophysiological functions of cell cycle proteins in post-mitotic neurons: implications for Alzheimer's disease. Acta Neuropathol (2015) 0.79
Pin1 levels are downregulated during ER stress in human neuroblastoma cells. Neurogenetics (2006) 0.79
Morphometric changes in the cortical microvascular network in Alzheimer's disease. J Alzheimers Dis (2010) 0.78
Activation of human microglia by fibrillar prion protein-related peptides is enhanced by amyloid-associated factors SAP and C1q. Neurobiol Dis (2005) 0.78
Apolipoprotein E and LRP1 Increase Early in Parkinson's Disease Pathogenesis. Am J Pathol (2011) 0.78
A new PERKspective on neurodegeneration. Sci Transl Med (2013) 0.78
Disturbed Ca2+ homeostasis increases glutaminyl cyclase expression; connecting two early pathogenic events in Alzheimer's disease in vitro. PLoS One (2012) 0.78
Neuroinflammation and common mechanism in Alzheimer's disease and prion amyloidosis: amyloid-associated proteins, neuroinflammation and neurofibrillary degeneration. Neurodegener Dis (2012) 0.77
Immunohistochemical characterization of novel monoclonal antibodies against the N-terminus of amyloid β-peptide. Amyloid (2013) 0.77