| scholarly article | Q13442814 |
| P819 | ADS bibcode | 2015PNAS..112.7821B |
| P356 | DOI | 10.1073/PNAS.1509744112 |
| P932 | PMC publication ID | 4485101 |
| P698 | PubMed publication ID | 26056265 |
| P50 | author | Gregory Petsko | Q5607114 |
| Dagmar Ringe | Q29840061 | ||
| P2093 | author name string | Xingli Li | |
| Eric J Huang | |||
| Yuxi Zhang | |||
| Steven Finkbeiner | |||
| Shulin Ju | |||
| Haiyan Qiu | |||
| Sami J Barmada | |||
| Elizabeth M H Tank | |||
| Arpana Arjun | |||
| Daniel Peisach | |||
| Hilary C Archbold | |||
| Anthony Batarse | |||
| P2860 | cites work | Molecular mechanisms for the RNA-dependent ATPase activity of Upf1 and its regulation by Upf2 | Q24297542 |
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| 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 | ||
| Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death | Q28287762 | ||
| Organizing principles of mammalian nonsense-mediated mRNA decay | Q28302705 | ||
| Characterization of Ighmbp2 in motor neurons and implications for the pathomechanism in a mouse model of human spinal muscular atrophy with respiratory distress type 1 (SMARD1) | Q28591190 | ||
| TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration | Q30493749 | ||
| Expansive Gene Transfer in the Rat CNS Rapidly Produces Amyotrophic Lateral Sclerosis Relevant Sequelae When TDP-43 is Overexpressed | Q30497589 | ||
| Senataxin, defective in the neurodegenerative disorder ataxia with oculomotor apraxia 2, lies at the interface of transcription and the DNA damage response | Q30532840 | ||
| Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice | Q30541711 | ||
| Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis. | Q30574944 | ||
| Automated microscope system for determining factors that predict neuronal fate. | Q30856679 | ||
| Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis | Q30986761 | ||
| 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 | ||
| TDP-43 regulates its mRNA levels through a negative feedback loop | Q33763346 | ||
| A yeast model of FUS/TLS-dependent cytotoxicity | Q33889537 | ||
| Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models. | Q33930825 | ||
| Rent1, a trans-effector of nonsense-mediated mRNA decay, is essential for mammalian embryonic viability | Q33930872 | ||
| Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice | Q34519947 | ||
| Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. | Q34982074 | ||
| ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation | Q35034350 | ||
| Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cells | Q35126200 | ||
| Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability | Q35889309 | ||
| Inhibition of nonsense-mediated mRNA decay (NMD) by a new chemical molecule reveals the dynamic of NMD factors in P-bodies | Q36119318 | ||
| A mutated human homologue to yeast Upf1 protein has a dominant-negative effect on the decay of nonsense-containing mRNAs in mammalian cells | Q36283006 | ||
| Association of the mammalian helicase MAH with the pre-mRNA splicing complex | Q36295319 | ||
| Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs | Q36650975 | ||
| Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis | Q37046148 | ||
| Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS. | Q37087752 | ||
| Molecular neuropathology of TDP-43 proteinopathies | Q37143159 | ||
| Mov10 and APOBEC3G localization to processing bodies is not required for virion incorporation and antiviral activity. | Q37252567 | ||
| ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects | Q37609639 | ||
| Pathogenic TARDBP mutations in amyotrophic lateral sclerosis and frontotemporal dementia: disease-associated pathways. | Q37810561 | ||
| Population based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology. | Q51457379 | ||
| MOV10 Is a 5' to 3' RNA helicase contributing to UPF1 mRNA target degradation by translocation along 3' UTRs. | Q54360570 | ||
| Huntington's Disease | Q83728053 | ||
| P4510 | describes a project that uses | ImageJ | Q1659584 |
| P433 | issue | 25 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | amyotrophic lateral sclerosis | Q206901 |
| P304 | page(s) | 7821-7826 | |
| P577 | publication date | 2015-06-08 | |
| P1433 | published in | Proceedings of the National Academy of Sciences of the United States of America | Q1146531 |
| P1476 | title | Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1. | |
| P478 | volume | 112 |
| Q89748818 | A native function for RAN translation and CGG repeats in regulating fragile X protein synthesis |
| Q93222043 | ALS mutations of FUS suppress protein translation and disrupt the regulation of nonsense-mediated decay |
| Q61135883 | Abnormal RNA stability in amyotrophic lateral sclerosis |
| Q64096518 | An Intramolecular Salt Bridge Linking TDP43 RNA Binding, Protein Stability, and TDP43-Dependent Neurodegeneration |
| Q63739888 | Automated four-dimensional long term imaging enables single cell tracking within organotypic brain slices to study neurodevelopment and degeneration |
| Q97069749 | C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression |
| Q37099358 | Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways |
| Q33721903 | Comprehensive whole genome sequence analyses yields novel genetic and structural insights for Intellectual Disability. |
| Q39323825 | Control of gene expression through the nonsense-mediated RNA decay pathway |
| Q93178436 | Disruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions |
| Q36173153 | Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity. |
| Q46308077 | E46K α-synuclein pathological mutation causes cell-autonomous toxicity without altering protein turnover or aggregation |
| Q99723633 | Functional genomics, genetic risk profiling and cell phenotypes in neurodegenerative disease |
| Q46236725 | Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein. |
| Q45056054 | Inhibition of nonsense-mediated RNA decay by ER stress |
| Q90411134 | Mating-based Overexpression Library Screening in Yeast |
| Q55688569 | Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization. |
| Q64911591 | Monitoring Neuronal Survival via Longitudinal Fluorescence Microscopy. |
| Q91767703 | Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization |
| Q58594611 | Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly |
| Q39005973 | Neurogenesis: Regulation by Alternative Splicing and Related Posttranscriptional Processes |
| Q58586716 | Nonsense-mediated RNA decay in the brain: emerging modulator of neural development and disease |
| Q96609560 | Nonsense-mediated mRNA decay factor UPF1 promotes aggresome formation |
| Q89720831 | Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity |
| Q33769272 | Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis |
| Q58621634 | Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain |
| Q34507318 | Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity. |
| Q47313248 | RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response |
| Q89146094 | RNA Degradation in Neurodegenerative Disease |
| Q30836611 | RNA sequencing of synaptic and cytoplasmic Upf1-bound transcripts supports contribution of nonsense-mediated decay to epileptogenesis. |
| Q38962112 | RNA-binding proteins implicated in neurodegenerative diseases |
| Q64089802 | Reactivation of nonsense-mediated mRNA decay protects against C9orf72 dipeptide-repeat neurotoxicity |
| Q92634114 | Splicing Players Are Differently Expressed in Sporadic Amyotrophic Lateral Sclerosis Molecular Clusters and Brain Regions |
| Q38685892 | Structural variants can be more informative for disease diagnostics, prognostics and translation than current SNP mapping and exon sequencing |
| Q50209927 | TDP43 and RNA instability in amyotrophic lateral sclerosis |
| Q55085029 | TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia. |
| Q64054855 | The RNA helicase UPF1 associates with mRNAs co-transcriptionally and is required for the release of mRNAs from gene loci |
| Q64768819 | The role of the protein-RNA recognition code in neurodegeneration |
| Q45304764 | The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin. |
| Q90027902 | Translation dysregulation in neurodegenerative disorders |
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