| review article | Q7318358 |
| scholarly article | Q13442814 |
| P6179 | Dimensions Publication ID | 1013099122 |
| P356 | DOI | 10.1007/S00401-013-1125-6 |
| P2888 | exact match | https://scigraph.springernature.com/pub.10.1007/s00401-013-1125-6 |
| P932 | PMC publication ID | 3661910 |
| P698 | PubMed publication ID | 23673820 |
| P5875 | ResearchGate publication ID | 236836908 |
| P50 | author | Leonard H van den Berg | Q56999697 |
| Jeroen Pasterkamp | Q64860284 | ||
| Ewout J N Groen | Q82478755 | ||
| Max Koppers | Q38324205 | ||
| P2093 | author name string | Anna M Blokhuis | |
| P2860 | cites work | UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis | Q47597072 |
| Contribution of TARDBP to Alzheimer's disease genetic etiology | Q48162352 | ||
| Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. | Q48183466 | ||
| Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord | Q48243077 | ||
| TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation | Q48255479 | ||
| Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human Disease | Q21131245 | ||
| ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B) | Q24294908 | ||
| Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes | Q24296835 | ||
| Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS | Q24297462 | ||
| Optineurin links myosin VI to the Golgi complex and is involved in Golgi organization and exocytosis | Q24300284 | ||
| Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules | Q24301077 | ||
| A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis | Q24304337 | ||
| Phosphorylation of the autophagy receptor optineurin restricts Salmonella growth | Q24304447 | ||
| Optineurin negatively regulates TNFalpha- induced NF-kappaB activation by competing with NEMO for ubiquitinated RIP | Q24337587 | ||
| Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia | Q24600027 | ||
| Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS | Q24608159 | ||
| Mixed tau, TDP-43 and p62 pathology in FTLD associated with a C9ORF72 repeat expansion and p.Ala239Thr MAPT (tau) variant | Q48342107 | ||
| Molecular analysis and biochemical classification of TDP-43 proteinopathy | Q48350651 | ||
| Novel deletion mutations of OPTN in amyotrophic lateral sclerosis in Japanese. | Q48623341 | ||
| A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study. | Q48791547 | ||
| Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. | Q49053200 | ||
| Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates. | Q50770142 | ||
| Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. | Q51774971 | ||
| High frequency of TARDBP gene mutations in Italian patients with amyotrophic lateral sclerosis. | Q51815155 | ||
| Vesicle associated membrane protein B (VAPB) is decreased in ALS spinal cord. | Q51887445 | ||
| TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis. | Q51920306 | ||
| Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death. | Q52537887 | ||
| Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies. | Q52710311 | ||
| Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies. | Q53299106 | ||
| Wide phenotypic spectrum of the TARDBP gene: homozygosity of A382T mutation in a patient presenting with amyotrophic lateral sclerosis, Parkinson's disease, and frontotemporal lobar degeneration, and in neurologically healthy subject. | Q54416684 | ||
| Progressive motor weakness in transgenic mice expressing human TDP-43. | Q54420401 | ||
| p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS | Q58477808 | ||
| Novel optineurin mutations in sporadic amyotrophic lateral sclerosis patients | Q59116555 | ||
| Molecular evaluation of human Ubiquilin 2 gene PXX domain in familial frontotemporal dementia patients | Q59543775 | ||
| Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders | Q59544757 | ||
| TAR-DNA Binding Protein 43 in Pick Disease | Q59549268 | ||
| Novel optineurin mutations in patients with familial and sporadic amyotrophic lateral sclerosis | Q60529641 | ||
| Analysis of OPTN as a causative gene for amyotrophic lateral sclerosis | Q82443570 | ||
| Screening of OPTN in French familial amyotrophic lateral sclerosis | Q83160690 | ||
| Optineurin in neurodegenerative diseases | Q83339684 | ||
| Optineurin is co-localized with FUS in basophilic inclusions of ALS with FUS mutation and in basophilic inclusion body disease | Q83426541 | ||
| Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism | Q84918163 | ||
| The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs | Q24622048 | ||
| Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS | Q24629495 | ||
| ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import | Q24630100 | ||
| Exome sequencing reveals VCP mutations as a cause of familial ALS | Q24631513 | ||
| A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions | Q24632492 | ||
| Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS | Q24633692 | ||
| A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD | Q24634583 | ||
| A new subtype of frontotemporal lobar degeneration with FUS pathology | Q24647697 | ||
| Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability | Q24648173 | ||
| Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans | Q27309039 | ||
| TDP-43-mediated neuron loss in vivo requires RNA-binding activity | Q27321280 | ||
| Hyperphosphorylation as a defense mechanism to reduce TDP-43 aggregation | Q27325625 | ||
| FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis | Q27339258 | ||
| FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration | Q27342067 | ||
| Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q28131672 | ||
| Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice | Q28131756 | ||
| Loss of autophagy in the central nervous system causes neurodegeneration in mice | Q28131804 | ||
| Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis | Q28131805 | ||
| Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis | Q28236796 | ||
| Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6 | Q28236805 | ||
| RNA granules: post-transcriptional and epigenetic modulators of gene expression | Q28245899 | ||
| TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling | Q28246694 | ||
| TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor | Q28261458 | ||
| TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis | Q28270779 | ||
| TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines | Q28284342 | ||
| Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis | Q28473603 | ||
| Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue | Q28475724 | ||
| Processing of optineurin in neuronal cells | Q28570083 | ||
| Delocalization of the multifunctional RNA splicing factor TLS/FUS in hippocampal neurones: exclusion from the nucleus and accumulation in dendritic granules and spine heads | Q28582163 | ||
| Generation and characterization of Sca2 (ataxin-2) knockout mice | Q28586415 | ||
| PolyQ repeat expansions in ATXN2 associated with ALS are CAA interrupted repeats | Q28741564 | ||
| Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels | Q29614781 | ||
| Mutations of optineurin in amyotrophic lateral sclerosis | Q29614836 | ||
| TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q29615597 | ||
| A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. | Q34882752 | ||
| Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. | Q34982074 | ||
| TDP-43 is recruited to stress granules in conditions of oxidative insult. | Q35003958 | ||
| The Ataxin-2 protein is required for microRNA function and synapse-specific long-term olfactory habituation. | Q35202757 | ||
| A patient carrying a homozygous p.A382T TARDBP missense mutation shows a syndrome including ALS, extrapyramidal symptoms, and FTD | Q35332219 | ||
| Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice | Q35551935 | ||
| On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia | Q35592875 | ||
| ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism | Q35605176 | ||
| A yeast functional screen predicts new candidate ALS disease genes | Q35641365 | ||
| Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span | Q35868223 | ||
| Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS | Q35887683 | ||
| Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability | Q35889309 | ||
| Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations | Q35979045 | ||
| An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity | Q35979060 | ||
| Mutant superoxide dismutase 1 (SOD1), a cause of amyotrophic lateral sclerosis, disrupts the recruitment of SMN, the spinal muscular atrophy protein to nuclear Cajal bodies | Q36083144 | ||
| Cdc37/Hsp90 protein complex disruption triggers an autophagic clearance cascade for TDP-43 protein | Q36098117 | ||
| Nuclear localization of human SOD1 and mutant SOD1-specific disruption of survival motor neuron protein complex in transgenic amyotrophic lateral sclerosis mice | Q36205839 | ||
| Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43 | Q36236376 | ||
| The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients. | Q36319626 | ||
| FUS-SMN protein interactions link the motor neuron diseases ALS and SMA | Q36354418 | ||
| Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models | Q36434528 | ||
| Repeat expansion in C9ORF72 in Alzheimer's disease | Q36441618 | ||
| Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis | Q36459963 | ||
| Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion | Q36464731 | ||
| Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis | Q36466787 | ||
| The FTD/ALS-associated RNA-binding protein TDP-43 regulates the robustness of neuronal specification through microRNA-9a in Drosophila | Q36477640 | ||
| Amyotrophic lateral sclerosis-frontotemporal lobar dementia in 3 families with p.Ala382Thr TARDBP mutations | Q36504551 | ||
| TDP-43 skeins show properties of amyloid in a subset of ALS cases | Q36508239 | ||
| TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis | Q36536399 | ||
| Tau pathology in frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansion | Q36553164 | ||
| C9ORF72 repeat expansions and other FTD gene mutations in a clinical AD patient series from Mayo Clinic | Q36576421 | ||
| Expression of human FUS protein in Drosophila leads to progressive neurodegeneration | Q36584516 | ||
| Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. | Q36602121 | ||
| Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions | Q34183099 | ||
| C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress | Q34206209 | ||
| FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations | Q34209561 | ||
| The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. | Q34263380 | ||
| Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination | Q34285952 | ||
| Roles of ataxin-2 in pathological cascades mediated by TAR DNA-binding protein 43 (TDP-43) and Fused in Sarcoma (FUS) | Q34304833 | ||
| Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules | Q34308339 | ||
| Knockdown of the Drosophila fused in sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches. | Q34314477 | ||
| The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. | Q34326849 | ||
| Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice | Q34519947 | ||
| Bunina bodies in amyotrophic lateral sclerosis | Q34585934 | ||
| Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness | Q34632269 | ||
| Male sterility and enhanced radiation sensitivity in TLS(-/-) mice | Q34662602 | ||
| Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice | Q34664325 | ||
| TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling | Q34829446 | ||
| A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity | Q36609145 | ||
| RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations | Q36626888 | ||
| Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. | Q36666933 | ||
| A pan-European study of the C9orf72 repeat associated with FTLD: geographic prevalence, genomic instability, and intermediate repeats | Q36801066 | ||
| ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules | Q36908224 | ||
| A genome-wide RNAi screen identifies genes regulating the formation of P bodies in C. elegans and their functions in NMD and RNAi | Q36922683 | ||
| TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: protein misfolding diseases without amyloidosis | Q36965517 | ||
| Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies | Q37136781 | ||
| Spinocerebellar ataxia 2 (SCA2). | Q37140787 | ||
| Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity | Q37164410 | ||
| Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. | Q37171556 | ||
| TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease | Q37179756 | ||
| Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo | Q37259106 | ||
| TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity | Q37339064 | ||
| TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration | Q37733168 | ||
| Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis | Q37806860 | ||
| TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration | Q37903412 | ||
| Making connections: pathology and genetics link amyotrophic lateral sclerosis with frontotemporal lobe dementia. | Q37929017 | ||
| Clinical diagnosis and management of amyotrophic lateral sclerosis | Q37944429 | ||
| Ubiquilins in the crosstalk among proteolytic pathways | Q38013079 | ||
| Atypical ubiquitylation - the unexplored world of polyubiquitin beyond Lys48 and Lys63 linkages. | Q38028343 | ||
| The genetics and neuropathology of amyotrophic lateral sclerosis | Q38035744 | ||
| TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration | Q38382616 | ||
| The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span. | Q38732625 | ||
| Polyubiquitin binding to optineurin is required for optimal activation of TANK-binding kinase 1 and production of interferon β. | Q38734490 | ||
| TDP-43 associates with stalled ribosomes and contributes to cell survival during cellular stress | Q39196751 | ||
| Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis | Q39240882 | ||
| The seeds of neurodegeneration: prion-like spreading in ALS | Q39247056 | ||
| A yeast model of optineurin proteinopathy reveals a unique aggregation pattern associated with cellular toxicity | Q39256992 | ||
| ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications | Q39319522 | ||
| p62/sequestosome 1 binds to TDP-43 in brains with frontotemporal lobar degeneration with TDP-43 inclusions | Q39335734 | ||
| Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase. | Q39372230 | ||
| Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations | Q39464446 | ||
| Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. | Q39584699 | ||
| RNA targets of wild-type and mutant FET family proteins | Q39603230 | ||
| Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations | Q39627330 | ||
| The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions. | Q39657178 | ||
| The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS). | Q39689378 | ||
| Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. | Q39761567 | ||
| Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo | Q39767215 | ||
| Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin | Q39838808 | ||
| Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43. | Q39839659 | ||
| Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells | Q39901141 | ||
| Ubiquitin-independent function of optineurin in autophagic clearance of protein aggregates | Q40710483 | ||
| Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid. | Q41341998 | ||
| Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases | Q41898768 | ||
| Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching | Q42096828 | ||
| Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43) | Q42110964 | ||
| Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. | Q42940304 | ||
| Genetic contribution of FUS to frontotemporal lobar degeneration | Q43177598 | ||
| Induction of amyloid fibrils by the C-terminal fragments of TDP-43 in amyotrophic lateral sclerosis | Q43200632 | ||
| Mutation within TARDBP leads to frontotemporal dementia without motor neuron disease. | Q43298473 | ||
| FUS mutations in familial amyotrophic lateral sclerosis in the Netherlands. | Q43506728 | ||
| Accumulation of insoluble forms of FUS protein correlates with toxicity in Drosophila | Q43719849 | ||
| Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues | Q43821139 | ||
| Novel TARDBP mutations in Nordic ALS patients | Q44606466 | ||
| Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology | Q44644427 | ||
| hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations | Q44742682 | ||
| The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases | Q45295489 | ||
| TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea. | Q45927072 | ||
| TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis. | Q46075183 | ||
| Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS). | Q46226347 | ||
| Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies | Q46396965 | ||
| Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS. | Q46461914 | ||
| Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations | Q46624207 | ||
| Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior | Q47071494 | ||
| TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis | Q29616311 | ||
| TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration | Q30490978 | ||
| Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6 | Q30492839 | ||
| TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration | Q30493749 | ||
| Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis | Q30494776 | ||
| Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy | Q30499509 | ||
| The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth. | Q30523244 | ||
| Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion | Q30532680 | ||
| ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. | Q30536540 | ||
| CDC7 inhibition blocks pathological TDP-43 phosphorylation and neurodegeneration. | Q30544943 | ||
| Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis. | Q30941385 | ||
| Transgenic rat model of neurodegeneration caused by mutation in the TDP gene. | Q33548806 | ||
| Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis | Q33649745 | ||
| TDP-43 is a developmentally regulated protein essential for early embryonic development | Q33673895 | ||
| Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy | Q33725391 | ||
| A Drosophila model for TDP-43 proteinopathy | Q33733651 | ||
| The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism | Q33786698 | ||
| Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS). | Q33799562 | ||
| Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS | Q33889532 | ||
| A yeast model of FUS/TLS-dependent cytotoxicity | Q33889537 | ||
| TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. | Q33932237 | ||
| Neuronal function and dysfunction of Drosophila dTDP | Q33932740 | ||
| TAR DNA-binding protein 43 in neurodegenerative disease | Q33941040 | ||
| Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons | Q33987984 | ||
| Neurotoxic effects of TDP-43 overexpression in C. elegans | Q34013543 | ||
| Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. | Q34044401 | ||
| ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. | Q34068067 | ||
| Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43. | Q34074385 | ||
| FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis | Q34118590 | ||
| Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. | Q34136342 | ||
| Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments | Q34137537 | ||
| TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor | Q34155890 | ||
| TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1. | Q34160352 | ||
| The aggregation and neurotoxicity of TDP-43 and its ALS-associated 25 kDa fragment are differentially affected by molecular chaperones in Drosophila | Q34182046 | ||
| P275 | copyright license | Creative Commons Attribution 2.0 Generic | Q19125117 |
| P6216 | copyright status | copyrighted | Q50423863 |
| P433 | issue | 6 | |
| P921 | main subject | cell | Q7868 |
| protein aggregation | Q7251455 | ||
| amyotrophic lateral sclerosis | Q206901 | ||
| P304 | page(s) | 777-794 | |
| P577 | publication date | 2013-05-15 | |
| 2013-06-01 | |||
| P1433 | published in | Acta Neuropathologica | Q343168 |
| P1476 | title | Protein aggregation in amyotrophic lateral sclerosis | |
| P478 | volume | 125 |
| Q64269328 | A Systematic Review of Suggested Molecular Strata, Biomarkers and Their Tissue Sources in ALS |
| Q64095142 | A context-based ABC model for literature-based discovery |
| Q38946004 | A novel p.E121G SOD1 mutation in slowly progressive form of amyotrophic lateral sclerosis induces cytoplasmic aggregates in cultured motor neurons and reduces cell viability |
| Q28392297 | ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease |
| Q36032446 | ALS mutations in TLS/FUS disrupt target gene expression |
| Q44216960 | ALS-associated protein FIG4 is localized in Pick and Lewy bodies, and also neuronal nuclear inclusions, in polyglutamine and intranuclear inclusion body diseases. |
| Q38998405 | AMPK in Neurodegenerative Diseases |
| Q42277252 | Adenoviral expression of TDP-43 and FUS genes and shRNAs for protein degradation pathways in rodent motoneurons in vitro and in vivo |
| Q58608836 | Aggregated SOD1 causes selective death of cultured human motor neurons |
| Q100958408 | Aggresome formation and liquid-liquid phase separation independently induce cytoplasmic aggregation of TAR DNA-binding protein 43 |
| Q64059609 | Alternative cleavage and polyadenylation of genes associated with protein turnover and mitochondrial function are deregulated in Parkinson's, Alzheimer's and ALS disease |
| Q56531746 | Amyotrophic lateral sclerosis |
| Q33796347 | Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms |
| Q27324123 | Amyotrophic lateral sclerosis-linked mutant VAPB inclusions do not interfere with protein degradation pathways or intracellular transport in a cultured cell model |
| Q38439914 | Animal models for prion-like diseases. |
| Q89964362 | Antioxidant Alternatives in the Treatment of Amyotrophic Lateral Sclerosis: A Comprehensive Review |
| Q26796599 | Autophagy and Neurodegeneration: Insights from a Cultured Cell Model of ALS |
| Q55405496 | Autophagy in Age-Associated Neurodegeneration. |
| Q46451525 | Beneficial effects of rapamycin in a Drosophila model for hereditary spastic paraplegia |
| Q30459373 | Biochemical Properties and Biological Functions of FET Proteins |
| Q49365640 | Biomarkers of Amyotrophic Lateral Sclerosis: Current Status and Interest of Oxysterols and Phytosterols. |
| Q59328934 | Blood Level of Glial Fibrillary Acidic Protein (GFAP) Does not Correlate With Disease Progression in a Rat Model of Familial ALS (SOD1 Transgenic) |
| Q33767845 | Blood biomarkers for amyotrophic lateral sclerosis: myth or reality? |
| Q26799233 | Brain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis? |
| Q35643260 | C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis |
| Q61052224 | Ca-activated K channels modulate microglia affecting motor neuron survival in hSOD1 mice |
| Q28084373 | Calcium dysregulation links ALS defective proteins and motor neuron selective vulnerability |
| Q48265316 | Causative Genes in Amyotrophic Lateral Sclerosis and Protein Degradation Pathways: a Link to Neurodegeneration. |
| Q64883214 | Chemical Basis of Reactive Oxygen Species Reactivity and Involvement in Neurodegenerative Diseases. |
| Q47222351 | Commentary: FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons |
| Q37099358 | Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways |
| Q42316145 | Components of a mammalian protein disaggregation/refolding machine are targeted to nuclear speckles following thermal stress in differentiated human neuronal cells. |
| Q64903704 | Could an Impairment in Local Translation of mRNAs in Glia be Contributing to Pathogenesis in ALS? |
| Q28116186 | Cryptic Amyloidogenic Elements in the 3' UTRs of Neurofilament Genes Trigger Axonal Neuropathy |
| Q37580967 | Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf's neurons and other sacral motor neurons. |
| Q37098891 | Developmentally Regulated RNA-binding Protein 1 (Drb1)/RNA-binding Motif Protein 45 (RBM45), a Nuclear-Cytoplasmic Trafficking Protein, Forms TAR DNA-binding Protein 43 (TDP-43)-mediated Cytoplasmic Aggregates |
| Q48061730 | Different aggregation states of a nuclear localization signal-tagged 25-kDa C-terminal fragment of TAR RNA/DNA-binding protein 43 kDa |
| Q39029166 | Disease-associated mutations of TDP-43 promote turnover of the protein through the proteasomal pathway |
| Q49335981 | Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis. |
| Q26753110 | Dysregulated axonal RNA translation in amyotrophic lateral sclerosis |
| Q38777893 | Efficacy of Stem Cell Therapy in Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-Analysis |
| Q97066870 | Exciting Complexity: The Role of Motor Circuit Elements in ALS Pathophysiology |
| Q38743399 | Exosome secretion is a key pathway for clearance of pathological TDP-43. |
| Q93059466 | Expression and Cellular Distribution of P-Glycoprotein and Breast Cancer Resistance Protein in Amyotrophic Lateral Sclerosis Patients |
| Q39041271 | Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity. |
| Q38712151 | FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination |
| Q33783811 | Formation and spreading of TDP-43 aggregates in cultured neuronal and glial cells demonstrated by time-lapse imaging |
| Q92084092 | Golgi Fragmentation in Neurodegenerative Diseases: Is There a Common Cause? |
| Q36211097 | Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences. |
| Q39037865 | High content analysis in amyotrophic lateral sclerosis |
| Q35702700 | Human immunodeficiency virus-associated dementia: a link between accumulation of viral proteins and neuronal degeneration. |
| Q103805257 | IL-10 based immunomodulation initiated at birth extends lifespan in a familial mouse model of amyotrophic lateral sclerosis |
| Q26766040 | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration |
| Q59591786 | In vivo evidence for reduced ion channel expression in motor axons of patients with amyotrophic lateral sclerosis |
| Q52726042 | Increased ROS Level in Spinal Cord of Wobbler Mice due to Nmnat2 Downregulation. |
| Q89982841 | Inflammatory profiles relate to survival in subtypes of amyotrophic lateral sclerosis |
| Q39361708 | Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis |
| Q39236779 | Interactions of pathological proteins in neurodegenerative diseases |
| Q41899271 | Intrathecal infusion of BMAA induces selective motor neuron damage and astrogliosis in the ventral horn of the spinal cord. |
| Q47108927 | Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease? |
| Q37133999 | Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins |
| Q97883105 | Lifestyle, Oxidative Stress, and Antioxidants: Back and Forth in the Pathophysiology of Chronic Diseases |
| Q33878853 | MTOR-independent, autophagic enhancer trehalose prolongs motor neuron survival and ameliorates the autophagic flux defect in a mouse model of amyotrophic lateral sclerosis. |
| Q36603298 | Macrophage-mediated inflammation and glial response in the skeletal muscle of a rat model of familial amyotrophic lateral sclerosis (ALS) |
| Q38272668 | MicroRNAs as potential circulating biomarkers for amyotrophic lateral sclerosis |
| Q57174649 | Minimotifs dysfunction is pervasive in neurodegenerative disorders |
| Q57153444 | Misfolded SOD1 pathology in sporadic Amyotrophic Lateral Sclerosis |
| Q60313606 | Motor and Nonmotor Symptoms of Parkinson’s Disease: Antagonistic Pleiotropy Phenomena Derived from α-Synuclein Evolvability? |
| Q48117832 | Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1C2-positive polyglutamine inclusions. |
| Q39428501 | Multifaceted role of SMCR8 as autophagy regulator. |
| Q93047576 | Mutant FUS and ELAVL4 (HuD) Aberrant Crosstalk in Amyotrophic Lateral Sclerosis |
| Q58594611 | Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly |
| Q47833173 | Mutation Screening of the CHCHD10 Gene in Chinese Patients with Amyotrophic Lateral Sclerosis |
| Q50530523 | Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons. |
| Q54261660 | NLRs as Helpline in the Brain: Mechanisms and Therapeutic Implications. |
| Q58737028 | Neurodegenerative Diseases: Regenerative Mechanisms and Novel Therapeutic Approaches |
| Q57289141 | Neuronal Cell Death Mechanisms in Major Neurodegenerative Diseases |
| Q26779424 | Neuronal response in Alzheimer's and Parkinson's disease: the effect of toxic proteins on intracellular pathways |
| Q90576491 | New molecular diagnostic trends and biomarkers for amyotrophic lateral sclerosis |
| Q38944792 | Next-generation sequencing in neuromuscular diseases |
| Q42284681 | Novel miR-b2122 regulates several ALS-related RNA-binding proteins. |
| Q89584199 | Novel therapeutic targets for amyotrophic lateral sclerosis |
| Q38829590 | Overexpression of Human SOD1 Leads to Discrete Defects in the Cerebellar Architecture in the Mouse |
| Q26801239 | Oxidative Stress in Neurodegenerative Diseases |
| Q35239387 | PPAR gamma activation is neuroprotective in a Drosophila model of ALS based on TDP-43. |
| Q89459649 | Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations |
| Q26828357 | Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders |
| Q38730474 | Pathology of the Aging Brain in Domestic and Laboratory Animals, and Animal Models of Human Neurodegenerative Diseases |
| Q52627910 | Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia. |
| Q47846742 | Phosphorylation and nuclear transit modulate the balance between normal function and terminal aggregation of the yeast RNA-binding protein Ssd1. |
| Q38789192 | Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins |
| Q41622472 | Plants-Derived Neuroprotective Agents: Cutting the Cycle of Cell Death through Multiple Mechanisms |
| Q47727180 | Possible concurrence of TDP-43, tau and other proteins in amyotrophic lateral sclerosis/frontotemporal lobar degeneration. |
| Q46294346 | Preventive and therapeutic potential of ascorbic acid in neurodegenerative diseases |
| Q28087798 | Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key? |
| Q33621868 | Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities? |
| Q48412910 | Proteomic profiling of the spinal cord in ALS: decreased ATP5D levels suggest synaptic dysfunction in ALS pathogenesis. |
| Q64975441 | Purinergic implication in amyotrophic lateral sclerosis-from pathological mechanisms to therapeutic perspectives. |
| Q38189807 | RNA metabolism in ALS: when normal processes become pathological. |
| Q38232795 | RNA-binding proteins associated molecular mechanisms of motor neuron degeneration pathogenesis |
| Q47569012 | RNA-binding proteins in neurodegeneration: mechanisms in aggregate |
| Q41325784 | Reactive Astrocytes Promote ALS-like Degeneration and Intracellular Protein Aggregation in Human Motor Neurons by Disrupting Autophagy through TGF-β1. |
| Q92932473 | Recent progress in the role of autophagy in neurological diseases |
| Q92796037 | Retinoic acid worsens ATG10-dependent autophagy impairment in TBK1-mutant hiPSC-derived motoneurons through SQSTM1/p62 accumulation |
| Q98184087 | Retromer stabilization results in neuroprotection in a model of Amyotrophic Lateral Sclerosis |
| Q46294217 | Rilmenidine promotes MTOR-independent autophagy in the mutant SOD1 mouse model of amyotrophic lateral sclerosis without slowing disease progression |
| Q38396129 | Role of MicroRNA Let-7 in Modulating Multifactorial Aspect of Neurodegenerative Diseases: an Overview. |
| Q89589416 | Role of Nuclear factor kappa B (NF-κB) Signalling in Neurodegenerative Diseases: An Mechanistic Approach |
| Q38453184 | S-nitrosylation of the thioredoxin-like domains of protein disulfide isomerase and its role in neurodegenerative conditions |
| Q98195450 | Serial MRI studies over 12 months using manual and atlas-based region of interest in patients with amyotrophic lateral sclerosis |
| Q30153414 | Solid-state NMR studies of metal-free SOD1 fibrillar structures |
| Q55287168 | State-of-the-Art Fluorescence Fluctuation-Based Spectroscopic Techniques for the Study of Protein Aggregation. |
| Q38341972 | Stem cells for amyotrophic lateral sclerosis modeling and therapy: myth or fact? |
| Q39236820 | Sulfated glycosaminoglycans in protein aggregation diseases |
| Q45233572 | Supersaturated proteins in ALS. |
| Q36260146 | TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis |
| Q34674858 | TDP-43 toxicity proceeds via calcium dysregulation and necrosis in aging Caenorhabditis elegans motor neurons |
| Q38969736 | TDP-43/FUS in motor neuron disease: Complexity and challenges. |
| Q47831395 | Targeting Cellular Stress Mechanisms and Metabolic Homeostasis by Chinese Herbal Drugs for Neuroprotection. |
| Q47301376 | Targeting the 26S Proteasome To Protect Against Proteotoxic Diseases |
| Q64075421 | Targeting the Sigma-1 Receptor via Pridopidine Ameliorates Central Features of ALS Pathology in a SOD1 Model |
| Q39396328 | The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains. |
| Q57471221 | The Emerging Role of DNA Damage in the Pathogenesis of the C9orf72 Repeat Expansion in Amyotrophic Lateral Sclerosis |
| Q33692255 | The Hsp70/Hsp90 Chaperone Machinery in Neurodegenerative Diseases |
| Q94561279 | The Peripheral Immune System and Amyotrophic Lateral Sclerosis |
| Q92404198 | The Role of Exercise as a Non-pharmacological Therapeutic Approach for Amyotrophic Lateral Sclerosis: Beneficial or Detrimental? |
| Q38701055 | The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis |
| Q92154033 | The activation of Mucolipin TRP channel 1 (TRPML1) protects motor neurons from L-BMAA neurotoxicity by promoting autophagic clearance |
| Q64089818 | The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight? |
| Q38882417 | The fused in sarcoma protein forms cytoplasmic aggregates in motor neurons derived from integration-free induced pluripotent stem cells generated from a patient with familial amyotrophic lateral sclerosis carrying the FUS-P525L mutation |
| Q90613923 | The multifaceted role of kinases in amyotrophic lateral sclerosis: genetic, pathological and therapeutic implications |
| Q104495311 | The neuroprotective effects of activated α7 nicotinic acetylcholine receptor against mutant copper-zinc superoxide dismutase 1-mediated toxicity |
| Q41143473 | The prion-like RNA-processing protein HNRPDL forms inherently toxic amyloid-like inclusion bodies in bacteria |
| Q55111742 | The proteome of neurofilament-containing protein aggregates in blood. |
| Q38212812 | The role of FUS gene variants in neurodegenerative diseases |
| Q37503447 | The role of gigaxonin in the degradation of the glial-specific intermediate filament protein GFAP. |
| Q64053733 | The role of neurofilament aggregation in neurodegeneration: lessons from rare inherited neurological disorders |
| Q34774553 | The ubiquitin C-terminal hydrolase L1 (UCH-L1) C terminus plays a key role in protein stability, but its farnesylation is not required for membrane association in primary neurons |
| Q90367656 | Traffic jam at the nuclear pore: All roads lead to nucleocytoplasmic transport defects in ALS/FTD |
| Q26782551 | UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration |
| Q38950512 | UPS Activation in the Battle Against Aging and Aggregation-Related Diseases: An Extended Review |
| Q30671325 | Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells. |
| Q92635935 | VAPB ER-Aggregates, A Possible New Biomarker in ALS Pathology |
| Q42316819 | Which TDP-43 aggregates are toxic in ALS? |
| Q38368814 | Zinc binding to RNA recognition motif of TDP-43 induces the formation of amyloid-like aggregates. |
| Q47579528 | mPOS is a novel mitochondrial trigger of cell death - implications for neurodegeneration |
| Q92817095 | p-Coumaric Acid Has Protective Effects against Mutant Copper-Zinc Superoxide Dismutase 1 via the Activation of Autophagy in N2a Cells |
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