Molecular mechanisms of promotion or suppression in renal tubular interstitial fibrosis

TRPS 1突变导致鼻咽综合征。Trps 1是肾单位发育所必需的，作用于Bmp 7的下游。由于Bmp 7抵消上皮间质转化（EMT）和逆转慢性肾损伤，我们研究了Trps 1在肾纤维化中的功能。实验结果表明，Trps 1缺陷增强TGF-β 1诱导的EMT和肾小管肠纤维化的调节量的Smad 7通过Arkadia/泛素介导的降解。

Mutations in TRPS1 cause tricho-rhino-pharyngeal syndrome. Trps1 is essential for nephron development, acting downstream of Bmp7. Because Bmp7 counteracts epithelial-to-mesenchymal transition(EMT) and reverses chronic renal injury, we examined the function of Trps1 in renal fibrosis. The results of the experiment suggest that Trps1 deficiency enhances TGF-b1-induced EMT and tubulointestinal fibrosis by modulating the amount of Smad7 through Arkadia/ubiquitin-mediated degradation.