Statins as a therapeutic approach for the treatment of pseudoxanthoma elasticum patients
- Pseudoxanthoma elasticum (PXE) is an autosomal recessive disorder caused by mutations in the \(\textit {ATP-binding cassette sub-family C member 6}\) gene. Our previous studies revealed that PXE might be associated with premature aging. Treatment with statins showed positive effects not only for PXE but also for other diseases associated with premature aging like Hutchinson–Gilford progeria syndrome. Nevertheless, the molecular mechanisms in the case of PXE remain unclear. Thus, this study was performed to evaluate the efficiency of atorvastatin by analyzing key characteristics of the PXE phenotype in primary human dermal fibroblasts of PXE patients. Our data indicate that an atorvastatin treatment has a positive effect, especially on factors associated with cholesterol biosynthesis and prenylation processes, whereas the effect on age- and calcification-related factors was less pronounced.
Author: | Janina TiemannGND, Christopher LindenkampGND, Ricarda PlümersORCiDGND, Isabel FaustORCiDGND, Cornelius KnabbeGND, Doris HendigORCiDGND |
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URN: | urn:nbn:de:hbz:294-82358 |
DOI: | https://doi.org/10.3390/cells10020442 |
Parent Title (English): | Cells |
Subtitle (English): | evaluation of the spectrum efficacy of atorvastatin in vitro |
Publisher: | MDPI |
Place of publication: | Basel |
Document Type: | Article |
Language: | English |
Date of Publication (online): | 2021/07/16 |
Date of first Publication: | 2021/02/19 |
Publishing Institution: | Ruhr-Universität Bochum, Universitätsbibliothek |
Tag: | atorvastatin; cholesterol biosynthesis; pseudoxanthoma elasticum |
Volume: | 10 |
Issue: | 2, Article 442 |
First Page: | 442-1 |
Last Page: | 442-15 |
Institutes/Facilities: | Herz- und Diabeteszentrum NRW, Institut für Laboratoriums- und Transfusionsmedizin |
open_access (DINI-Set): | open_access |
Licence (English): | Creative Commons - CC BY 4.0 - Attribution 4.0 International |