Research map: Targeting TDP-43 in sporadic amyotrophic lateral sclerosis

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Papers in this map

  1. Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis · Manuela Neumann · 2006 · 6764 citations · Cited by this paper
  2. Recent advances in mass spectrometry methods for the investigation of oligomeric intermediates of amyloid protein aggregation · Carlo Santambrogio · 2026 · Cites this paper
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  6. Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43 · Magdalini Polymenidou · 2011 · 1399 citations · Cited by this paper
  7. Clinical outcomes at 6 months after MRgFUS thalamotomy in Parkinson’s disease: a large longitudinal cohort study · 2026 · Related
  8. Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS · Andrew C. Elden · 2010 · 1366 citations · Cited by this paper
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  10. Prognostic factors in ALS: A critical review · Adriano Chiò · 2009 · 1186 citations · Cited by this paper
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  12. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2 · Stefan-M. Pulst · 1996 · 1131 citations · Cited by this paper
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  14. Stress granules as crucibles of ALS pathogenesis · Yun R. Li · 2013 · 992 citations · Cited by this paper
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  16. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial · Koji Abe · 2017 · 950 citations · Cited by this paper
  17. Use of blood-based neurofilament light chain as an endpoint in clinical trials of neurodegenerative conditions: a scoping review · 2026 · Related
  18. TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD · Jonathan P. Ling · 2015 · 758 citations · Cited by this paper
  19. Long-term risk of dementia following encephalitis: a large-scale retrospective cohort study of electronic health records · 2026 · Related
  20. Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration · Ze’ev Melamed · 2019 · 693 citations · Cited by this paper
  21. Bi-allelic intermediate ATXN2 repeat expansions are associated with slow progressing, leg-onset familial ALS · 2026 · Related
  22. TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD · Ching‐Chieh Chou · 2018 · 639 citations · Cited by this paper
  23. The TAK1–NLK–MAPK-related pathway antagonizes signalling between β-catenin and transcription factor TCF · Tohru Ishitani · 1999 · 624 citations · Cited by this paper
  24. A proposal for new diagnostic criteria for ALS · Jeremy M. Shefner · 2020 · 617 citations · Cited by this paper
  25. TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration · Hans Wils · 2010 · 612 citations · Cited by this paper
  26. TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A · X. Rosa Ma · 2022 · 610 citations · Cited by this paper
  27. Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice · Lindsay A. Becker · 2017 · 602 citations · Cited by this paper
  28. Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis · Sami J. Barmada · 2010 · 586 citations · Cited by this paper
  29. Phase 1–2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS · Jonathan S. Mill · 2020 · 567 citations · Cited by this paper
  30. TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A · Anna‐Leigh Brown · 2022 · 553 citations · Cited by this paper
  31. Trial of Sodium Phenylbutyrate–Taurursodiol for Amyotrophic Lateral Sclerosis · Sabrina Paganoni · 2020 · 544 citations · Cited by this paper
  32. Mutation of FIG4 causes neurodegeneration in the pale tremor mouse and patients with CMT4J · Clement Y. Chow · 2007 · 522 citations · Cited by this paper
  33. Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice · Lionel Muller Igaz · 2011 · 458 citations · Cited by this paper
  34. Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models · Sami J. Barmada · 2014 · 443 citations · Cited by this paper
  35. Deleterious Variants of FIG4, a Phosphoinositide Phosphatase, in Patients with ALS · Clement Y. Chow · 2009 · 407 citations · Cited by this paper
  36. TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development · Chantelle F. Sephton · 2009 · 378 citations · Cited by this paper
  37. Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43 · Adam K. Walker · 2015 · 365 citations · Cited by this paper
  38. The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons · Wenzhang Wang · 2013 · 343 citations · Cited by this paper
  39. TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response · Peng Wang · 2019 · 301 citations · Cited by this paper

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