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Reactive Oxygen Species and Oxidative Stress in the Pathogenesis and Progression of Genetic Diseases of the Connective Tissue
Antioxidants ( IF 6.0 ) Pub Date : 2020-10-19 , DOI: 10.3390/antiox9101013
Gustavo Egea , Francesc Jiménez-Altayó , Victoria Campuzano

Connective tissue is known to provide structural and functional “glue” properties to other tissues. It contains cellular and molecular components that are arranged in several dynamic organizations. Connective tissue is the focus of numerous genetic and nongenetic diseases. Genetic diseases of the connective tissue are minority or rare, but no less important than the nongenetic diseases. Here we review the impact of reactive oxygen species (ROS) and oxidative stress on the onset and/or progression of diseases that directly affect connective tissue and have a genetic origin. It is important to consider that ROS and oxidative stress are not synonymous, although they are often closely linked. In a normal range, ROS have a relevant physiological role, whose levels result from a fine balance between ROS producers and ROS scavenge enzymatic systems. However, pathology arises or worsens when such balance is lost, like when ROS production is abnormally and constantly high and/or when ROS scavenge (enzymatic) systems are impaired. These concepts apply to numerous diseases, and connective tissue is no exception. We have organized this review around the two basic structural molecular components of connective tissue: The ground substance and fibers (collagen and elastic fibers).

中文翻译:

结缔组织遗传病发病机理和进展中的活性氧和氧化应激

已知结缔组织为其他组织提供结构和功能上的“胶水”特性。它包含以若干动态组织排列的细胞和分子成分。结缔组织是许多遗传和非遗传疾病的焦点。结缔组织的遗传疾病为少数或罕见,但同非遗传疾病同样重要。在这里,我们回顾了活性氧(ROS)和氧化应激对直接影响结缔组织并具有遗传起源的疾病的发作和/或进展的影响。重要的是要考虑到ROS和氧化应激不是同义词,尽管它们通常紧密相连。在正常范围内,ROS具有相关的生理作用,其水平是由于ROS生产者与ROS清除酶系统之间的良好平衡而产生的。然而,当失去这种平衡时,病理学就会出现或恶化,例如当ROS产生异常且持续高和/或ROS清除(酶)系统受损时。这些概念适用于多种疾病,结缔组织也不例外。我们围绕结缔组织的两个基本结构分子组成组织了本篇综述:地面物质和纤维(胶原蛋白和弹性纤维)。
更新日期:2020-10-19
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