Dysregulation of store-operated calcium entry in fibroblast lines from adult and juvenile-onset Huntington’s disease patients
The mHTT upregulates SOCE in adult-onset HD fibroblasts and downregulates it in juvenile-onset HD fibroblast lines; however, SOCE levels do not correlate with the length of CAG repeats encoding mHTT. Despite opposing trends compared to age-related controls, similar levels of SOCE in both HD-onset fibroblasts were detected. Both C<sub>20</sub>H<sub>22</sub>BrClN<sub>2</sub> and EVP4593 show potential for stabilizing SOCE in adult-onset HD. These findings suggest that dysregulated SOCE could be investigated as a peripheral target for studying pathological processes potentially associated with Huntington's disease.
Authors
- Alžbeta Mühlbäck (ORCID: https://orcid.org/0000-0002-0385-3071)
- Magdalena Czeredys (ORCID: https://orcid.org/0000-0001-5419-1234)
- Ewelina Latoszek (ORCID: https://orcid.org/0000-0002-7883-6989)
- H Hansíková
- Jacek Kuźnicki (ORCID: https://orcid.org/0000-0001-6486-0657)
- Jiří Klempíř
- Georg Bernhard Landwehrmeyer
- Samuel Oluwafemi Egbuwalo
Institutions
- Universität Ulm (DE)
- Charles University (CZ)
- Isar-Amper-Klinikum München-Ost (DE)
- International Institute of Molecular and Cell Biology (PL)
- Mossakowski Medical Research Institute, Polish Academy of Sciences (PL)
- General University Hospital in Prague (CZ)
Publication Details
- Journal
- Pharmacological Reports
- Published
- 2026-01-20
- DOI
- https://doi.org/10.1007/s43440-025-00820-8
- Primary Topic
- Genetic Neurodegenerative Diseases
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Ministerstvo Zdravotnictví Ceské Republiky