Ghanian Zahra, Maleki Sepideh, Park SunYoung, Sorenson Christine M, Sheibani Nader, Ranji Mahsa
Department of Electrical Engineering, University of Wisconsin Milwaukee, Milwaukee, WI, USA.
J Biophotonics. 2014 Oct;7(10):799-809. doi: 10.1002/jbio.201300033. Epub 2013 Jun 6.
Hereditary Hemorrhagic Telangiectasia-1 (HHT-1) is a vascular disease caused by mutations in the endoglin (Eng)/CD105 gene. The objective of this study was to quantify the oxidative state of a rodent model of HHT-1 using an optical imaging technique. We used a cryofluorescence imaging instrument to quantitatively assess tissue metabolism in this model. Mitochondrial redox ratio (FAD/NADH), FAD RR, was used as a quantitative marker of the metabolic status and was examined in the kidneys, and eyes of wild-type and Eng +/- mice. Kidneys and eyes from wild-type P21, 6W, and 10M old mice showed, respectively, a 9% (±2), 24% (±0.4), 15% (±1), and 23% (±4), 33% (±0.6), and 30% (±2) change in the mean FAD RR compared to Eng +/- mice at the same age. Thus, endoglin haploinsufficiency is associated with less oxidative stress in various organs and mitigation of angiogenesis.
遗传性出血性毛细血管扩张症1型（HHT-1）是一种由内皮糖蛋白（Eng）/CD105基因突变引起的血管疾病。本研究的目的是使用光学成像技术量化HHT-1啮齿动物模型的氧化状态。我们使用低温荧光成像仪器定量评估该模型中的组织代谢。线粒体氧化还原比（FAD/NADH），即FAD RR，用作代谢状态的定量标志物，并在野生型和Eng +/-小鼠的肾脏和眼睛中进行检测。与相同年龄的Eng +/-小鼠相比，野生型P21、6周和10月龄小鼠的肾脏和眼睛平均FAD RR分别有9%（±2）、24%（±0.4）、15%（±1）以及23%（±4）、33%（±0.6）和30%（±2）的变化。因此，内皮糖蛋白单倍体不足与各器官中较低的氧化应激以及血管生成的减轻有关。
