Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons

Abstract Nuclear clearance and cytoplasmic aggregation of TDP-43, initially identified in ALS-FTD, are hallmark pathological features observed across a spectrum of neurodegenerative diseases. We previously found that TDP-43 loss-of-function leads to transcriptome-wide inclusion of deleterious crypti...

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Main Authors: Irika R. Sinha, Parker S. Sandal, Holly Spence, Grace D. Burns, Aswathy Peethambaran Mallika, Fatemeh Abbasinejad, Katherine E. Irwin, Anna Lourdes F. Cruz, Vania Wang, Shaelyn R. Marx, Josué Llamas Rodríguez, Ben Langmead, Jenna M. Gregory, Philip C. Wong, Jonathan P. Ling
Format: Article
Language:English
Published: Nature Portfolio 2025-07-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-025-62004-5
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author Irika R. Sinha
Parker S. Sandal
Holly Spence
Grace D. Burns
Aswathy Peethambaran Mallika
Fatemeh Abbasinejad
Katherine E. Irwin
Anna Lourdes F. Cruz
Vania Wang
Shaelyn R. Marx
Josué Llamas Rodríguez
Ben Langmead
Jenna M. Gregory
Philip C. Wong
Jonathan P. Ling
author_facet Irika R. Sinha
Parker S. Sandal
Holly Spence
Grace D. Burns
Aswathy Peethambaran Mallika
Fatemeh Abbasinejad
Katherine E. Irwin
Anna Lourdes F. Cruz
Vania Wang
Shaelyn R. Marx
Josué Llamas Rodríguez
Ben Langmead
Jenna M. Gregory
Philip C. Wong
Jonathan P. Ling
author_sort Irika R. Sinha
collection DOAJ
description Abstract Nuclear clearance and cytoplasmic aggregation of TDP-43, initially identified in ALS-FTD, are hallmark pathological features observed across a spectrum of neurodegenerative diseases. We previously found that TDP-43 loss-of-function leads to transcriptome-wide inclusion of deleterious cryptic exons, a signature detected in presymptomatic biofluids and postmortem ALS-FTD brain tissue, but the upstream mechanisms that lead to TDP-43 dysregulation remain unclear. Here, we developed a web-based resource (SnapMine) to determine the levels of TDP-43 cryptic exon inclusion across hundreds of thousands of publicly available RNA sequencing datasets. We established cryptic exon inclusion levels across a variety of human cells and tissues to provide ground truth references for future studies on TDP-43 dysregulation. We then explored studies that were entirely unrelated to TDP-43 or neurodegeneration and found that ciclopirox olamine (CPX), an FDA-approved antifungal, can trigger the inclusion of TDP-43-associated cryptic exons in a variety of mouse and human primary cells. CPX induction of cryptic exons arises from heavy metal toxicity and oxidative stress, suggesting that similar vulnerabilities could play a role in neurodegeneration. Our work demonstrates how diverse datasets can be linked through common biological features and underscores how public archives of sequencing data remain a vastly underutilized resource with tremendous potential for uncovering novel insights into complex biological mechanisms and diseases.
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spelling doaj-art-78c8a4350f4a4fd89550df129be3a4672025-08-20T03:05:10ZengNature PortfolioNature Communications2041-17232025-07-0116111510.1038/s41467-025-62004-5Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exonsIrika R. Sinha0Parker S. Sandal1Holly Spence2Grace D. Burns3Aswathy Peethambaran Mallika4Fatemeh Abbasinejad5Katherine E. Irwin6Anna Lourdes F. Cruz7Vania Wang8Shaelyn R. Marx9Josué Llamas Rodríguez10Ben Langmead11Jenna M. Gregory12Philip C. Wong13Jonathan P. Ling14Department of Neuroscience, Johns Hopkins University School of MedicineDepartment of Pathology, Johns Hopkins University School of MedicineInstitute of Medical Sciences, University of AberdeenDepartment of Pathology, Johns Hopkins University School of MedicineDepartment of Pathology, Johns Hopkins University School of MedicineScientific Software Engineering Center, Johns Hopkins UniversityDepartment of Neuroscience, Johns Hopkins University School of MedicineDepartment of Neuroscience, Johns Hopkins University School of MedicineDepartment of Oncology, Johns Hopkins University School of MedicineDepartment of Pathology, Johns Hopkins University School of MedicineDepartment of Neuroscience, Johns Hopkins University School of MedicineKavli Neuroscience Discovery Institute, Johns Hopkins UniversityInstitute of Medical Sciences, University of AberdeenDepartment of Neuroscience, Johns Hopkins University School of MedicineDepartment of Pathology, Johns Hopkins University School of MedicineAbstract Nuclear clearance and cytoplasmic aggregation of TDP-43, initially identified in ALS-FTD, are hallmark pathological features observed across a spectrum of neurodegenerative diseases. We previously found that TDP-43 loss-of-function leads to transcriptome-wide inclusion of deleterious cryptic exons, a signature detected in presymptomatic biofluids and postmortem ALS-FTD brain tissue, but the upstream mechanisms that lead to TDP-43 dysregulation remain unclear. Here, we developed a web-based resource (SnapMine) to determine the levels of TDP-43 cryptic exon inclusion across hundreds of thousands of publicly available RNA sequencing datasets. We established cryptic exon inclusion levels across a variety of human cells and tissues to provide ground truth references for future studies on TDP-43 dysregulation. We then explored studies that were entirely unrelated to TDP-43 or neurodegeneration and found that ciclopirox olamine (CPX), an FDA-approved antifungal, can trigger the inclusion of TDP-43-associated cryptic exons in a variety of mouse and human primary cells. CPX induction of cryptic exons arises from heavy metal toxicity and oxidative stress, suggesting that similar vulnerabilities could play a role in neurodegeneration. Our work demonstrates how diverse datasets can be linked through common biological features and underscores how public archives of sequencing data remain a vastly underutilized resource with tremendous potential for uncovering novel insights into complex biological mechanisms and diseases.https://doi.org/10.1038/s41467-025-62004-5
spellingShingle Irika R. Sinha
Parker S. Sandal
Holly Spence
Grace D. Burns
Aswathy Peethambaran Mallika
Fatemeh Abbasinejad
Katherine E. Irwin
Anna Lourdes F. Cruz
Vania Wang
Shaelyn R. Marx
Josué Llamas Rodríguez
Ben Langmead
Jenna M. Gregory
Philip C. Wong
Jonathan P. Ling
Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
Nature Communications
title Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
title_full Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
title_fullStr Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
title_full_unstemmed Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
title_short Large-scale RNA-Seq mining reveals ciclopirox olamine induces TDP-43 cryptic exons
title_sort large scale rna seq mining reveals ciclopirox olamine induces tdp 43 cryptic exons
url https://doi.org/10.1038/s41467-025-62004-5
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