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.

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Metadaten
Author:Janina TiemannGND, Christopher LindenkampGND, Ricarda PlümersORCiDGND, Isabel FaustORCiDGND, Cornelius KnabbeGND, Doris HendigORCiDGND
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):License LogoCreative Commons - CC BY 4.0 - Attribution 4.0 International