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Record W4415384313 · doi:10.1038/s41593-025-02050-w

TDP-43 loss induces cryptic polyadenylation in ALS/FTD

2025· article· en· W4415384313 on OpenAlexaff
Sam Bryce-Smith, Anna‐Leigh Brown, Max Z. Y. J. Chien, Dario Dattilo, Puja R. Mehta, Francesca Mattedi, Simone Barattucci, Alla Mikheenko, Matteo Zanovello, Flaminia Pellegrini, Sara Emad El-Agamy, Matthew Yome, Sarah E. Hill, Yue Qi, Kai Sun, Eugeni Ryadnov, Yixuan Wan, Hemali Phatnani, Justin Kwan, Dhruv Sareen, James R. Broach, Zachary Simmons, Ximena Arcila-Londono, Edward B. Lee, Vivianna M. Van Deerlin, Neil A. Shneider, Ernest Fraenkel, Lyle W. Ostrow, Frank Baas, Noah Zaitlen, James D. Berry, Andrea Malaspina, Gregory A. Cox, Leslie M. Thompson, Steven Finkbeiner, Efthimios Dardiotis, Timothy M. Miller, Siddharthan Chandran, Suvankar Pal, Eran Hornstein, Daniel J. MacGowan, Terry Heiman‐Patterson, Molly Hammell, Nikolaos A. Patsopoulos, Josh Dubnau, Avindra Nath, Robert Bowser, Matthew B. Harms, Eleonora Aronica, Mary Poss, Jennifer E. Phillips‐Cremins, John F. Crary, Nazem Atassi, Dale J. Lange, Darius J. Adams, Leonidas Stefanis, Marc Gotkine, Robert H. Baloh, Suma Babu, Sabrina Paganoni, Ophir Shalem, Colin Smith, Bin Zhang, Thomas G. Blanchard, Brent T. Harris, Iris Broce, Vivian E. Drory, John Ravits, Corey T. McMillan, Vilas Menon, Lani F. Wu, Steven J. Altschuler, Yossef Lerner, Rita Sattler, Kendall Van Keuren‐Jensen, Orit Rozenblatt–Rosen, Kerstin Lindblad‐Toh, Katharine Nicholson, Peter K. Gregersen, Jeong‐Ho Lee, Oleg Butovsky, Matt Brauer, T. Nickerson, Shameek Biswas, Kimberly Wilson, Sulev Kõks, Stephen Muljo, Bryan J. Traynor, Robert Moccia, Seng H. Cheng, Andrew Deubler, Giovanni Coppola, Mickey Atwal, Michael Cantor, William Salerno, Eli A. Stahl, Matt Anderson, David Frendewey, Daphne Koller, Mary Rozenman, Jose Norberto S. Vargas, Nicol Birsa, Towfique Raj, Jack Humphrey, Matthew J. Keuss, Oscar G. Wilkins, Michael E. Ward, Maria Secrier, Pietro Fratta

Bibliographic record

VenueNature Neuroscience · 2025
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsInstitute of Aging
FundersNational Institute on AgingNational Institute of Neurological Disorders and StrokeMedical Research CouncilRosetrees TrustMotor Neurone Disease AssociationBrain Research UKWellcome TrustFrancis Crick InstituteNational Institutes of HealthCancer Research UKTarget ALS
KeywordsPolyadenylationSpecies complexRNAFrontotemporal dementiaUntranslated regionRNA-binding proteinNuclear poreRNA splicing

Abstract

fetched live from OpenAlex

Nuclear depletion and cytoplasmic aggregation of the RNA-binding protein TDP-43 are cellular hallmarks of amyotrophic lateral sclerosis (ALS). TDP-43 nuclear loss causes de-repression of cryptic exons, yet cryptic alternative polyadenylation (APA) events have been largely overlooked. In this study, we developed a bioinformatic pipeline to reliably identify alternative last exons, 3' untranslated region (3'UTR) extensions and intronic polyadenylation APA event types, and we identified cryptic APA sites induced by TDP-43 loss in induced pluripotent stem cell (iPSC)-derived neurons. TDP-43 binding sites are enriched at sites of these cryptic events, and TDP-43 can both repress and enhance APA. All categories of cryptic APA were also identified in ALS and frontotemporal dementia (FTD) postmortem brain tissue. RNA sequencing (RNA-seq), thiol(SH)-linked alkylation for the metabolic sequencing of RNA (SLAM-seq) and ribosome profiling (Ribo-seq) revealed that distinct cryptic APA categories have different downstream effects on transcript levels and that cryptic 3'UTR extensions can increase RNA stability, leading to increased translation. In summary, we demonstrate that TDP-43 nuclear depletion induces cryptic APA, expanding the palette of known consequences of TDP-43.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.919
Threshold uncertainty score0.508

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.002
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.019
GPT teacher head0.357
Teacher spread0.338 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations19
Published2025
Admission routes1
Has abstractyes

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