An alteration in cell/matrix interactions is one of the suggested mechanisms leading to cyst formation in polycystic kidney diseases. Most of these interactions are mediated by beta 1-integrins, a subfamily of integrin receptors, formed by the association of the beta 1-chain with different alpha-subunits. To date, no study on alpha-integrin subunit distribution during the early stages of cyst development has been reported. Using immunofluorescence, we analyzed the distribution of alpha-integrin subunits (alpha 1, alpha 2, alpha 3, alpha 5, and alpha 6) and basement membrane proteins in kidneys of fetuses with autosomal dominant (ADPKD) or autosomal recessive polycystic kidney disease (ARPKD). The distribution was compared with that observed in normal fetal and post-natal kidneys, and in fetal cystic dysplasia and Meckel syndrome. Marked increase in alpha 1-integrin staining was observed in normal and cystic collecting duct cells of both polycystic diseases (PKD), compared with normal and cystic controls. The distribution of integrin subunits alpha 2, alpha 3, and alpha 6 was irregular in cyst epithelial cells of PKD and cystic controls. The increased expression of the alpha 1-subunit specifically observed in PKD collecting duct cells may be an early consequence of the genetic defect in ARPKD. In ADPKD it parallels the reported expression of polycystin, the protein product of PKD1. The irregular expression of alpha 2, alpha 3, and alpha 6 integrin subunits observed in all types of cysts suggests that cell/matrix interactions are altered early and may participate in the development of cysts, perhaps by contributing to the deregulation of cell survival in cystic diseases.

译文

细胞/基质相互作用的改变是导致多囊肾疾病中囊肿形成的建议机制之一。这些相互作用中的大多数是由 β1-整合素 (一种整合素受体的亚家族) 介导的,由 β1-链与不同的 α-亚基结合形成。迄今为止,尚无关于囊肿发育早期阶段的 α-整联蛋白亚基分布的研究。使用免疫荧光,我们分析了常染色体显性遗传 (ADPKD) 或常染色体隐性多囊肾 (ARPKD) 胎儿肾脏中 α-整合素亚基 (α1,α2,α3,α5和 α6) 和基底膜蛋白的分布。将分布与正常胎儿和产后肾脏以及胎儿囊性发育不良和梅克尔综合征的分布进行了比较。与正常和囊性对照相比,在两种多囊性疾病 (PKD) 的正常和囊性集合管细胞中观察到 α1-整合素染色显着增加。在PKD和囊性对照的囊肿上皮细胞中,整合素亚基 α2,α3和 α6的分布不规则。在PKD收集导管细胞中特异性观察到的 α1亚基表达增加可能是ARPKD遗传缺陷的早期结果。在ADPKD中,它与pkd1的蛋白质产物多囊蛋白的报道表达相似。在所有类型的囊肿中观察到的 α2,α3和 α6整联蛋白亚基的不规则表达表明,细胞/基质相互作用在早期发生改变,并可能参与囊肿的发展,这可能是通过改变囊性疾病中细胞存活的失调。

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