Nephrology Division, Internal Medicine Department, Hospital General Juan Cardona, c/ Pardo Bazán s/n, 15406, Ferrol, Spain.
University of Santiago de Compostela Medical School, Santiago de Compostela, Spain.
Mol Biol Rep. 2021 May;48(5):4865-4878. doi: 10.1007/s11033-021-06478-8. Epub 2021 Jun 15.
Elastic fibers are essential components of the arterial extracellular matrix. They consist of the protein elastin and an array of microfibrils that support the protein and connect it to the surrounding matrix. The elastin gene encodes tropoelastin, a protein that requires extensive cross-linking to become elastin. Tropoelastin is expressed throughout human life, but its expression levels decrease with age, suggesting that the potential to synthesize elastin persists during lifetime although declines with aging. The initial abnormality documented in human atherosclerosis is fragmentation and loss of the elastic network in the medial layer of the arterial wall, suggesting an imbalance between elastic fiber injury and restoration. Damaged elastic structures are not adequately repaired by synthesis of new elastic elements. Progressive collagen accumulation follows medial elastic fiber disruption and fibrous plaques are formed, but advanced atherosclerosis lesions do not develop in the absence of prior elastic injury. Aging is associated with arterial extracellular matrix anomalies that evoke those present in early atherosclerosis. The reduction of elastic fibers with subsequent collagen accumulation leads to arterial stiffening and intima-media thickening, which are independent predictors of incident hypertension in prospective community-based studies. Arterial stiffening precedes the development of hypertension. The fundamental role of the vascular elastic network to arterial structure and function is emphasized by congenital disorders caused by mutations that disrupt normal elastic fiber production. Molecular changes in the genes coding tropoelastin, lysyl oxidase (tropoelastin cross-linking), and elastin-associated microfibrils, including fibrillin-1, fibulin-4, and fibulin-5 produce severe vascular injury due to absence of functional elastin.
弹性纤维是动脉细胞外基质的重要组成部分。它们由弹性蛋白和一系列微纤维组成,这些微纤维支撑着弹性蛋白并将其与周围的基质连接起来。弹性蛋白基因编码原弹性蛋白,一种需要广泛交联才能成为弹性蛋白的蛋白质。原弹性蛋白在人类生命的各个阶段都有表达,但随着年龄的增长,其表达水平会下降,这表明在整个生命周期中都有合成弹性蛋白的潜力,尽管随着年龄的增长会下降。人类动脉粥样硬化中最早记录到的异常是动脉壁中膜层弹性网络的碎片化和丧失,这表明弹性纤维损伤和修复之间存在失衡。受损的弹性结构不能通过新的弹性元素合成得到充分修复。随后,胶原的积累会导致中膜层的弹性纤维断裂,形成纤维斑块,但如果没有先前的弹性损伤,就不会形成晚期动脉粥样硬化病变。衰老与动脉细胞外基质异常有关,这些异常与早期动脉粥样硬化中存在的异常相似。随着弹性纤维的减少和随后的胶原积累,导致动脉变硬和内膜-中层增厚,这是前瞻性社区研究中高血压事件的独立预测因素。动脉僵硬先于高血压的发生。血管弹性网络对动脉结构和功能的基本作用,被由突变引起的先天性疾病所强调,这些突变会破坏正常弹性纤维的产生。编码原弹性蛋白、赖氨酰氧化酶(原弹性蛋白交联)和弹性蛋白相关微纤维的基因的分子变化,包括原纤维蛋白-1、纤维连接蛋白-4 和纤维连接蛋白-5,由于缺乏功能性弹性蛋白,会导致严重的血管损伤。