| P50 | author | Daisuke Ito | Q30430138 |
| | Norihiro Suzuki | Q30430157 |
| P2093 | author name string | Mami Hatano | |
| P2860 | cites work | Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS | Q24297462 |
| | Characterization of amyotrophic lateral sclerosis-linked P56S mutation of vesicle-associated membrane protein-associated protein B (VAPB/ALS8) | Q24299071 |
| | Phosphorylation of the autophagy receptor optineurin restricts Salmonella growth | Q24304447 |
| | p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy | Q24312147 |
| | Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia | Q24323876 |
| | C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking | Q24337586 |
| | Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia | Q24600027 |
| | Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis | Q24605104 |
| | Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS | Q24608159 |
| | Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis | Q24619298 |
| | Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function | Q24629019 |
| | 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 |
| | Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS | Q24633692 |
| | Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. | Q42940304 |
| | Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis. | Q44491457 |
| | Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology | Q44644427 |
| | Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis | Q45232740 |
| | A critical role for amino-terminal glutamine/asparagine repeats in the formation and propagation of a yeast prion | Q46347783 |
| | Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA. | Q46628586 |
| | Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations | Q46986726 |
| | TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics | Q47939995 |
| | Mutations in the 3' untranslated region of FUS causing FUS overexpression are associated with amyotrophic lateral sclerosis. | Q48021519 |
| | [(18)F]Flutemetamol PET imaging and cortical biopsy histopathology for fibrillar amyloid β detection in living subjects with normal pressure hydrocephalus: pooled analysis of four studies | Q48342095 |
| | Neuronal uptake of tau/pS422 antibody and reduced progression of tau pathology in a mouse model of Alzheimer's disease | Q48624391 |
| | Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice | Q48645659 |
| | C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD. | Q48794622 |
| | Phenotype of matrin-3-related distal myopathy in 16 German patients. | Q51701990 |
| | Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. | Q52607544 |
| | A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation. | Q53368534 |
| | SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis | Q55671566 |
| | A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD | Q24634583 |
| | Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS | Q24634807 |
| | Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis | Q26863439 |
| | State of play in amyotrophic lateral sclerosis genetics | Q26864816 |
| | The role of D-serine and glycine as co-agonists of NMDA receptors in motor neuron degeneration and amyotrophic lateral sclerosis (ALS) | Q27003135 |
| | SKP1 connects cell cycle regulators to the ubiquitin proteolysis machinery through a novel motif, the F-box | Q27936367 |
| | Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q28131672 |
| | 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 |
| | Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis | Q28237200 |
| | Welander distal myopathy is caused by a mutation in the RNA-binding protein TIA1 | Q28285385 |
| | TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia | Q28294001 |
| | Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue | Q28475724 |
| | Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization | Q28588090 |
| | TBK-1 Promotes Autophagy-Mediated Antimicrobial Defense by Controlling Autophagosome Maturation | Q29030353 |
| | A C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagy | Q29465530 |
| | Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels | Q29614781 |
| | Mutations of optineurin in amyotrophic lateral sclerosis | Q29614836 |
| | Stress granules: the Tao of RNA triage | Q29615263 |
| | TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q29615597 |
| | Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein | Q29619232 |
| | The C9orf72 repeat expansion disrupts nucleocytoplasmic transport. | Q29871482 |
| | Autosomal-dominant distal myopathy associated with a recurrent missense mutation in the gene encoding the nuclear matrix protein, matrin 3 | Q30438210 |
| | TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration | Q30493749 |
| | Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion | Q30532680 |
| | Prion-like transmission of protein aggregates in neurodegenerative diseases | Q33544162 |
| | TDP-43 is a developmentally regulated protein essential for early embryonic development | Q33673895 |
| | Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase | Q33842154 |
| | Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism | Q34136356 |
| | Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress | Q34157304 |
| | 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 |
| | C9ORF72 Regulates Stress Granule Formation and Its Deficiency Impairs Stress Granule Assembly, Hypersensitizing Cells to Stress | Q38781435 |
| | Disturbance of proteasomal and autophagic protein degradation pathways by amyotrophic lateral sclerosis-linked mutations in ubiquilin 2. | Q38788893 |
| | Prion-like properties of pathological TDP-43 aggregates from diseased brains | Q39129644 |
| | Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. | Q39584699 |
| | RNA targets of wild-type and mutant FET family proteins | Q39603230 |
| | FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis | Q39927199 |
| | FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization | Q41950782 |
| | Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43) | Q42110964 |
| | Ubiquilin at a crossroads in protein degradation pathways | Q42152457 |
| | C9orf72 is required for proper macrophage and microglial function in mice | Q42364302 |
| | Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS. | Q42693496 |
| | Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1. | Q42818503 |
| | Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS. | Q42925023 |
| | Roles of ataxin-2 in pathological cascades mediated by TAR DNA-binding protein 43 (TDP-43) and Fused in Sarcoma (FUS) | Q34304833 |
| | The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. | Q34326849 |
| | Profilin 1 associates with stress granules and ALS-linked mutations alter stress granule dynamics. | Q34424308 |
| | GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport | Q34491033 |
| | Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. | Q34982074 |
| | TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord | Q35260685 |
| | Genome-wide association study identifies variants at CSF1, OPTN and TNFRSF11A as genetic risk factors for Paget's disease of bone | Q35552797 |
| | Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways | Q35624305 |
| | A yeast functional screen predicts new candidate ALS disease genes | Q35641365 |
| | Defective Proteasome Delivery of Polyubiquitinated Proteins by Ubiquilin-2 Proteins Containing ALS Mutations | Q35663774 |
| | Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration. | Q35776562 |
| | Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism | Q35853004 |
| | ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43. | Q35886826 |
| | Exome sequencing identifies FUS mutations as a cause of essential tremor | Q36152927 |
| | TDP-43 is intercellularly transmitted across axon terminals | Q36310560 |
| | ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function | Q36320469 |
| | Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits | Q36407722 |
| | ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function | Q36548825 |
| | C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits | Q36555249 |
| | TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD. | Q36781316 |
| | Stress granules as crucibles of ALS pathogenesis. | Q36804082 |
| | CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia | Q36809438 |
| | Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss | Q36906420 |
| | TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: protein misfolding diseases without amyloidosis | Q36965517 |
| | 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 | Q37164667 |
| | Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. | Q37171556 |
| | Stages of pTDP-43 pathology in amyotrophic lateral sclerosis | Q37202139 |
| | Antibody uptake into neurons occurs primarily via clathrin-dependent Fcγ receptor endocytosis and is a prerequisite for acute tau protein clearance. | Q37368709 |
| | C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins | Q37476206 |
| | ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration | Q37593829 |
| | ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arbor | Q37650180 |
| | TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration | Q37733168 |
| | Tau immunotherapy for Alzheimer's disease | Q38407801 |
| | Development of Passive Immunotherapies for Synucleinopathies | Q38679627 |
| P433 | issue | 415 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | amyotrophic lateral sclerosis | Q206901 |
| | neurodegeneration | Q1755122 |
| P577 | publication date | 2017-11-01 | |
| P1433 | published in | Science Translational Medicine | Q1573955 |
| P1476 | title | RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration | |
| P478 | volume | 9 | |