Patient-derived iPSC models suggest astrocytic involvement in DM1 neuropathology

Myotonic dystrophy type 1 (DM1) is caused by a CTG-repeat expansion in the DMPKgene, producing toxic RNA that sequesters MBNL proteins and disrupts RNA splicing. In addition to muscular symptoms, DM1 causes cognitive impairments, suggesting central nervous system involvement. We model these effects using patient-derived iPSC-derived neurons (iNeurons) and astrocytes (hAstrocytes).

Authors

Derick G. Wansink (ORCID: https://orcid.org/0000-0002-6773-8662)
Hans van Bokhoven (ORCID: https://orcid.org/0000-0002-2153-9254)
Lisa Rahm (ORCID: https://orcid.org/0000-0003-1420-9027)
Marieke Willemse
Anton M. Simoes (ORCID: https://orcid.org/0009-0001-1823-7802)

Institutions

Radboud University Nijmegen (NL)
University Medical Center (US)
Radboud University Medical Center (NL)

Publication Details

Journal: Zenodo (CERN European Organization for Nuclear Research)
Published: 2026-06-17
DOI: https://doi.org/10.5281/zenodo.20728748
Primary Topic: Genetic Neurodegenerative Diseases
Type: article
Field-Weighted Citation Impact: 0.00

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article

Patient-derived iPSC models suggest astrocytic involvement in DM1 neuropathology

Derick G. Wansink, Hans van Bokhoven, Lisa Rahm, Marieke Willemse et al.

Jun 17, 2026

Zenodo (CERN European Organization for Nuclear Research)

Genetic Neurodegenerative Diseases

article

Patient-derived iPSC models suggest astrocytic involvement in DM1 neuropathology

Derick G. Wansink, Hans van Bokhoven, Lisa Rahm, Marieke Willemse, Anton M. Simoes

June 17, 2026article en

Abstract

Myotonic dystrophy type 1 (DM1) is caused by a CTG-repeat expansion in the DMPKgene, producing toxic RNA that sequesters MBNL proteins and disrupts RNA splicing. In addition to muscular symptoms, DM1 causes cognitive impairments, suggesting central nervous system involvement. We model these effects using patient-derived iPSC-derived neurons (iNeurons) and astrocytes (hAstrocytes).

Zenodo (CERN European Organization for Nuclear Research)

Radboud University Nijmegen (NL), University Medical Center (US), Radboud University Medical Center (NL)

Good health and well-being

Openalex Percentile: Top 11%

Genetic Neurodegenerative Diseases

DOI Open Access PDF OpenAlex

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