دورية أكاديمية

Autoimmune disease mouse model exhibits pulmonary arterial hypertension.

التفاصيل البيبلوغرافية
العنوان: Autoimmune disease mouse model exhibits pulmonary arterial hypertension.
المؤلفون: Koichi Sugimoto, Kazuhiko Nakazato, Akihiko Sato, Satoshi Suzuki, Akiomi Yoshihisa, Takeshi Machida, Shu-Ichi Saitoh, Hideharu Sekine, Yasuchika Takeishi
المصدر: PLoS ONE, Vol 12, Iss 9, p e0184990 (2017)
بيانات النشر: Public Library of Science (PLoS), 2017.
سنة النشر: 2017
المجموعة: LCC:Medicine
LCC:Science
مصطلحات موضوعية: Medicine, Science
الوصف: Pulmonary arterial hypertension is often associated with connective tissue disease. Although there are some animal models of pulmonary hypertension, an autoimmune disease-based model has not yet been reported. MRL/lpr mice, which have hypergammaglobulinemia, produce various autoimmune antibodies, and develop vasculitis and nephritis spontaneously. However, little is known about pulmonary circulation in these mice. In the present study, we examined the pulmonary arterial pressure in MRL/lpr mice.We used female MRL/lpr mice aged between 12 and 14 weeks. Fluorescent immunostaining showed that there was no deposition of immunoglobulin or C3 in the lung tissue of the MRL/lpr mice. Elevation of interferon-γ and interleukin-6 was recognized in the lung tissue of the MRL/lpr mice. Right ventricular systolic pressure, Fulton index and the ratio of right ventricular weight to body weight in the MRL/lpr mice were significantly higher than those in wild type mice with same background (C57BL/6). The medial smooth muscle area and the proportion of muscularized vessels in the lung tissue of the MRL/lpr mice were larger than those of the C57BL/6 mice. Western blot analysis demonstrated markedly elevated levels of prepro-endothelin-1 and survivin as well as decreased endothelial nitric oxide synthase phosphorylation in the lung tissue of the MRL/lpr mice. Terminal deoxynucleotidyl-transferase-mediated dUTP nick end-labeling assay showed the resistance against apoptosis of pulmonary arterial smooth muscle cells in the MRL/lpr mice.We showed that MRL/lpr mice were complicated with pulmonary hypertension. MRL/lpr mice appeared to be a useful model for studying the mechanism of pulmonary hypertension associated with connective tissue diseases.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1932-6203
العلاقة: http://europepmc.org/articles/PMC5605000?pdf=renderTest; https://doaj.org/toc/1932-6203Test
DOI: 10.1371/journal.pone.0184990
الوصول الحر: https://doaj.org/article/b4699cc942304e6e94937c993827f43fTest
رقم الانضمام: edsdoj.b4699cc942304e6e94937c993827f43f
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:19326203
DOI:10.1371/journal.pone.0184990