他汀类药物作为治疗弹性假黄瘤患者的治疗方法：阿托伐他汀体外疗效谱的评估。

Institut für Laboratoriums- und Transfusionsmedizin, Herz- und Diabeteszentrum Nordrhein-Westfalen, Universitätsklinik der Ruhr-Universität Bochum, 32545 Bad Oeynhausen, Germany.

Cells. 2021 Feb 19;10(2):442. doi: 10.3390/cells10020442.

Pseudoxanthoma elasticum (PXE) is an autosomal recessive disorder caused by mutations in the 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.

弹性假黄瘤（PXE）是一种常染色体隐性遗传病，由基因突变引起。我们之前的研究表明，PXE 可能与早衰有关。他汀类药物治疗不仅对 PXE 而且对其他与早衰相关的疾病（如 Hutchinson-Gilford 早老综合征）都有积极的影响。然而，PXE 病例的分子机制尚不清楚。因此，本研究通过分析 PXE 患者原代人真皮成纤维细胞的 PXE 表型的关键特征，评估阿托伐他汀的疗效。我们的数据表明，阿托伐他汀治疗有积极的效果，特别是对与胆固醇生物合成和 prenylation 过程相关的因素，而对与年龄和钙化相关的因素的影响则不那么明显。