Mitochondrial toxins cause widespread downregulation of pathways in X-linked dystonia-parkinsonism patient-derived neurons
Karen Grütz, Axel Künstner, Christin Krause, Letizia Santinelli, Sören Franzenburg, Jenny Ghelfi, Anne Grünewald, Raymond L Rosales, Norbert Brüggemann, Hauke Busch, Christine Klein, Philip Seibler · 2026
Read Mitochondrial toxins cause widespread downregulation of pathways in X-linked dystonia-parkinsonism patient-derived neurons by Karen Grütz, Axel Künstner, Christin Krause, Letizia Santinelli, Sören Franzenburg, Jenny Ghelfi, Anne Grünewald, Raymond L Rosales, Norbert Brüggemann, Hauke Busch, Christine Klein, Philip Seibler (2026) in the Tower of Babel, a free online library of public-domain works preserved in clean, searchable text. No sign-up. The full text is served as a plain-text file at a predictable URL, where text is present: https://antarctic-labs.com/tower-of-babel/pmc-13261877.txt.
Description
Karen Grütz and co-authors study a rare movement disorder in a 2026 paper on patient-derived neurons. The disorder, X-linked dystonia-parkinsonism, stems from an inserted stretch of DNA in a gene that helps start gene reading. The team grew neurons from patients and compared their gene activity with controls, at rest and under mitochondrial toxins.
At rest, patient neurons mostly ran their pathways hotter than controls. The toxins flipped that picture: pathways shut down broadly, covering genome upkeep, gene control, and adaptive nerve function. DNA damage also rose. The authors read the patient neurons as unusually fragile under energy stress. That fragility, they suggest, may help explain how the disease unfolds.
Catalog record
| CREATOR | Karen Grütz, Axel Künstner, Christin Krause, Letizia Santinelli, Sören Franzenburg, Jenny Ghelfi, Anne Grünewald, Raymond L Rosales, Norbert Brüggemann, Hauke Busch, Christine Klein, Philip Seibler |
|---|---|
| YEAR | 2026 |
| COLLECTION | PAPERS |
| FORMAT | TXT |
| FILE SIZE | 32 KB |
| VERSION | 1.0 |
| LICENSE | CC BY 4.0 |
| RIGHTS | CC BY |
| SOURCE | Europe PMC |