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首页> 外文期刊>Folia neuropathologica >Disturbed integrin expression in the vascular media in CADASIL
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Disturbed integrin expression in the vascular media in CADASIL

机译：CADASIL中血管介质中整合素表达异常

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CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an inherited angiopathy characterized by degeneration and loss of vascular smooth muscle cells (VSMCs) of still unknown pathomechanism. Many functions of VSMCs, such as adhesion, apoptosis, contraction, differentiation, migration, and proliferation are determined by integrins - surface adhesion receptors involved in binding and interactions between cells and extracellular matrix (ECM). Since integrins play such an important role in VSMCs biology, disturbances in their expression may influence myocytes behavior and fate in CADASIL In this study, we focused on the most important compounds of VSMCs integrins: subunits alpha(4), beta 1, and beta(3) in an attempt to elucidate their immune expression in the arterial media of CADASIL patients. The immunohistochemistry revealed a decreased expression of integrin beta 1 subunit (p < 0.001) but similar to the control expression of integrin subunits alpha(4) and beta(3). Decreased beta 1 immunoreactivity was observed in capillary vessels, arterioles, and small arteries. The abnormal immune expression of integrin beta 1 subunit was found even in microvessels without microscopically noted degenerative changes, which suggests that this is an early phenomenon in CADASIL Since integrin beta 1 subunit is a compound of 10 heterodimer integrin receptors, its disturbed expression may significantly influence VSMCs biology leading to myocytes degeneration and loss via anoikis a type of apoptotic cell death due to loss or inappropriate cell adhesion to ECM.
机译：CADASIL（伴有皮层下梗塞和白质脑病的常染色体显性遗传性脑病）是一种遗传性血管病，其特征是尚不清楚病机机制的血管平滑肌细胞（VSMC）的变性和丧失。 VSMC的许多功能，例如粘附，凋亡，收缩，分化，迁移和增殖，都由整合素决定-整合素是表面粘附受体，参与细胞与细胞外基质（ECM）的结合和相互作用。由于整联蛋白在VSMC生物学中起着如此重要的作用，因此其表达失调可能会影响CADASIL中的心肌细胞行为和命运。在这项研究中，我们集中于VSMC整联蛋白最重要的化合物：亚基alpha（4），β1和beta（ 3）试图阐明其在CADASIL患者动脉介质中的免疫表达。免疫组化显示整合素β1亚基的表达降低（p <0.001），但与整合素亚基alpha（4）和beta（3）的对照表达相似。在毛细血管，小动脉和小动脉中观察到β1免疫反应性降低。甚至在没有显微镜下观察到的变性变化的微血管中也发现了整联蛋白β1亚基的异常免疫表达，这表明这是CADASIL的早期现象，因为整联蛋白β1亚基是10个异二聚体整联蛋白受体的化合物，其受干扰的表达可能会显着影响VSMCs的生物学行为通过无神经导致肌细胞变性和丢失，这是由于丢失或不适当的细胞对ECM粘附导致的一种凋亡细胞死亡。

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