ArticleNature neuroscience2018
TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD.
Erratum issuedOpen access · greenAbstract read
In one paragraph
Article in Nature neuroscience, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 162 papers.
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162citing papers in PubMed
22.9field-weighted citation impact, top 1% of its field
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Who cites it
162 citing papers in PubMed, 293 citations in OpenAlex.
- Human TDP-43 expression worsens FTD-related phenotypes in progranulin-insufficient mice.Neurobiology of disease · 2026Article
- Using Machine Learning to Automate the Analysis of an Olfactory Habituation-Dishabituation Task in Mice.Brain and behavior · 2026Article
- TDP-43 proteinopathy as a biomarker and therapeutic target in amyotrophic lateral sclerosis.Biochemical Society transactions · 2026Review
- TDP-43 Aggregation: The Healthy-Toxic Balance of the Prion-Like Domain.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Review
- ALS-FTD-linked CCNFJournal of neuroinflammation · 2026Article
- Predictive Cellular Signatures from Live Human Motor Neurons Distinguish TDP-43 ALS and Enable ALS Subtype Stratification.bioRxiv : the preprint server for biology · 2026Article
- TDP-43 pathology is linked to motor neuron loss and is independent of stress granulesbioRxiv : the preprint server for biology · 2026Article
- Review
- TDP43 and hnRNP K Regulate Alternative Splicing of DNAJC5.Cell biology international · 2026Article
- Splicing the narrative: alternative TARDBP splicing and its relation to neurodegeneration in ALS and FTD.The Journal of clinical investigation · 2026Review
- BLOC1S1 depletion via splice-switching oligonucleotides improves mitochondrial respiration and rescues ALS phenotypes.Molecular therapy : the journal of the American Society of Gene Therapy · 2026Article
- Inhibiting Glycogen Synthase Kinase 3 Suppresses TDP-43-Mediated Neurotoxicity in a Caspase-Dependent Manner.Molecular neurobiology · 2026Article
- Reduction of RAD23A extends lifespan and mitigates pathology in a mouse model of TDP-43 proteinopathy.Nature communications · 2026Article
- Experimental Models and Translational Strategies in Neuroprotective Drug Development with Emphasis on Alzheimer's Disease.Molecules (Basel, Switzerland) · 2026Review
- A partial deletion of the Tardbp 3'UTR affects TDP-43 regulation and leads to motor dysfunction in mice.Experimental animals · 2026Article
- Human iPSC-derived motor neurons as a platform for elucidating TDP-43-related amyotrophic lateral sclerosis pathogenesis: a mini review.Frontiers in molecular neuroscience · 2026Review
- TARDBP (TDP-43) Knock-in Zebrafish Display a Late-Onset Motor Phenotype and Loss of Large Spinal Cord Motor Neurons.Annals of neurology · 2026Article
- Dysregulated lactate metabolism synergizes with ALS genetic risk factors to accelerate motor decline.PloS one · 2026Article
- Synaptic changes contribute to persistent extra-motor behaviour deficits in amyotrophic lateral sclerosis.Acta neuropathologica communications · 2025Article
- CRISPR/Cas9 a genomic engineering technology for treatment in ALS mouse models.Regenerative therapy · 2025Review
102 more citing papers are in PubMed but not listed here.
Corrections and comments
- Erratum issued
Authors and funding
23 authors at 11 institutions in 4 countries.
Matthew A White *The Babraham Institute, Cambridge, UK.
Eosu Kim *Department of Psychology and MRC/Wellcome Trust Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, UK.
Amanda DuffyDepartment of Neuroscience, Brown University, Providence, RI, USA.
Robert AdalbertJohn van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK.
Benjamin U PhillipsDepartment of Psychology and MRC/Wellcome Trust Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, UK.
Owen M PetersThe Vollum Institute, Oregon Health & Science University, Portland, OR, USA.
Jodie StephensonSheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK.
Sujeong YangJohn van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK.
Francesca MassenzioThe Babraham Institute, Cambridge, UK.
Ziqiang LinThe Babraham Institute, Cambridge, UK.
Simon AndrewsThe Babraham Institute, Cambridge, UK.
Anne Segonds-PichonThe Babraham Institute, Cambridge, UK.
Jake MettervilleDepartment of Neurology, UMass Medical School, Worcester, MA, USA.
Lisa M SaksidaDepartment of Psychology and MRC/Wellcome Trust Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, UK.
Richard MeadSheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK.
Richard R RibchesterSBMS, University of Edinburgh, Edinburgh, UK.
Youssef BarhomiDepartment of Cognitive, Linguistic and Psychological Sciences, Brown University, Providence, RI, USA.
Thomas SerreDepartment of Cognitive, Linguistic and Psychological Sciences, Brown University, Providence, RI, USA.
Michael P ColemanThe Babraham Institute, Cambridge, UK.
Justin R FallonDepartment of Neuroscience, Brown University, Providence, RI, USA.
Timothy J BusseyDepartment of Psychology and MRC/Wellcome Trust Behavioural and Clinical Neuroscience Institute, University of Cambridge, Cambridge, UK.
Robert H BrownDepartment of Neurology, UMass Medical School, Worcester, MA, USA.
Jemeen SreedharanThe Babraham Institute, Cambridge, UK. jemeen.sreedharan@kcl.ac.uk.ORCID http://orcid.org/0000-0002-8682-6844
Babraham Institute · GBBrown University · USUniversity of Cambridge · GBUniversity of Massachusetts Chan Medical School · USWestern University · CAOregon Health & Science University · USQueen Mary University of London · GBUniversity of Edinburgh · GBUniversity of Sheffield · GBWellcome/MRC Cambridge Stem Cell Institute · GBYonsei University · KR
Funding
Identification of genes causative for familial ALS gene using exome sequencingR01NS073873 · NINDS · UNIV OF MASSACHUSETTS MED SCH WORCESTER · PI LANDERS, JOHN E · 2011 to 2019
$4.6MCharacterization of KIFAP3, a Modifier of Survival in Sporadic ALSR01NS065847 · NINDS · UNIV OF MASSACHUSETTS MED SCH WORCESTER · PI LANDERS, JOHN E · 2010 to 2014
$1.8MPhase 2 Study of L-Serine in HSN Type 1R01FD004127 · FDA · MASSACHUSETTS GENERAL HOSPITAL · PI EICHLER, FLORIAN S · 2012 to 2016
$1.6MSilencing C9or72 with rAAV Mediated RNAiR01NS088689 · NINDS · UNIV OF MASSACHUSETTS MED SCH WORCESTER · PI BROWN, ROBERT H., MUELLER, CHRISTIAN · 2014 to 2017
$1.6MBrain Science Computer ClusterS10OD016366 · OD · BROWN UNIVERSITY · PI DONOGHUE, JOHN P · 2013 to 2013
$600kFDA HHS R01 FD004127Medical Research Council MR/K010611/1Medical Research Council MR/L003813/1Motor Neurone Disease Association RIBCHESTER/APR15/838-791Motor Neurone Disease Association SITRAN/APR13/983-797Motor Neurone Disease Association SITRAN/JUL16/987-797Motor Neurone Disease Association SREEDHARAN/APR16/849-791Motor Neurone Disease Association SREEDHARAN/JAN13/943-795National Centre for the Replacement, Refinement and Reduction of Animals in Research NC/N001451/1NIH HHS S10 OD016366NINDS NIH HHS R01 NS065847NINDS NIH HHS R01 NS073873NINDS NIH HHS R01 NS088689
Abstract
Amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) constitutes a devastating disease spectrum characterized by 43-kDa TAR DNA-binding protein (TDP-43) pathology. Understanding how TDP-43 contributes to neurodegeneration will help direct therapeutic efforts. Here we have created a TDP-43 knock-in mouse with a human-equivalent mutation in the endogenous mouse Tardbp gene. TDP-43
Indexed as
Amyotrophic Lateral SclerosisAnimalsBrainChoice BehaviorCognition DisordersConditioning, OperantDementiaDisease Models, AnimalDNA-Binding ProteinsFemaleGene Expression RegulationMaleMemory DisordersMiceMice, Inbred C57BLMice, TransgenicDNA-Binding ProteinsTardbp protein, mouse
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.