PDF(Pubmed)
Abstract：
Kidney failures in infants are mostly caused by congenital anomalies of the kidney and urinary tract (CAKUT), which are among the most common congenital birth disorders worldwide when paired with cardiac abnormalities. People with CAKUT often have severe kidney failure as a result of a wide range of abnormalities that can occur alone or in conjunction with other syndromic disorders. In this study, we aimed to investigate the expression pattern of CAKUT candidate genes alpha-8 integrin (ITGA8) and Van Gogh-like 2 (VANGL2) in fetal tissues of healthy and CAKUT-affected kidneys using immunohistochemistry and immunofluorescence. We found that under CAKUT circumstances, the expressions of ITGA8 and VANGL2 are changed. Additionally, we showed that VANGL2 expression is constant during fetal aging, but ITGA8 expression varies. Moreover, compared to normal healthy kidneys (CTRL), ITGA8 is poorly expressed in duplex kidneys (DKs) and dysplastic kidneys (DYS), whereas VANGL2 is substantially expressed in dysplastic kidneys (DYS) and poorly expressed in hypoplastic kidneys (HYP). These results point to VANGL2 and ITGA8 as potential prognostic indicators for CAKUT malformations. Further research is necessary to explore the molecular mechanisms underlying this differential expression of ITGA8 and VANGL2.
摘要：
婴儿的肾功能衰竭主要由先天性肾脏和泌尿道异常（CAKUT）引起。与心脏异常配对时，这是全球最常见的先天性出生疾病之一。CAKUT患者通常由于广泛的异常而导致严重的肾衰竭，这些异常可以单独发生或与其他综合征一起发生。在这项研究中,我们旨在通过免疫组织化学和免疫荧光法研究CAKUT候选基因α-8整合素(ITGA8)和梵高样2(VANGL2)在健康和CAKUT感染肾脏的胎儿组织中的表达模式.我们发现在CAKUT的情况下,ITGA8和VANGL2的表达式被改变。此外,我们表明VANGL2表达在胎儿衰老过程中是恒定的，但ITGA8表达不同。此外，与正常健康的肾脏（CTRL）相比，ITGA8在双重肾脏（DKs）和发育不良肾脏（DYS）中表达不良，而VANGL2在发育不良的肾脏（DYS）中大量表达，而在发育不良的肾脏（HYP）中表达很少。这些结果表明VANGL2和ITGA8是CAKUT畸形的潜在预后指标。需要进一步的研究来探索ITGA8和VANGL2差异表达的分子机制。
