Program in Developmental and Stem Cell Biology, The Hospital for Sick Children, Toronto Medical Discovery Tower, 101 College Street, Toronto, Ontario, M5G 1L7, Canada.
Pediatr Nephrol. 2011 Sep;26(9):1365-71. doi: 10.1007/s00467-010-1704-x. Epub 2010 Dec 15.
The kidney is the most common site of congenital malformations that result in impaired renal function. Yet, the molecular mechanisms that control renal malformations are poorly understood. The Hedgehog signaling pathway plays critical roles during mammalian organogenesis. Aberrant Hedgehog signaling results in severe congenital abnormalities, including renal malformations. Here, we review the current body of knowledge on Hedgehog signaling during renal morphogenesis and highlight the gaps in our understanding. Furthermore, we propose mechanisms by which Hedgehog signaling contributes to both normal and abnormal renal development.
肾脏是导致肾功能受损的先天性畸形最常见的部位。然而,控制肾脏畸形的分子机制还知之甚少。Hedgehog 信号通路在哺乳动物器官发生中发挥着关键作用。异常的 Hedgehog 信号会导致严重的先天性异常,包括肾脏畸形。在这里,我们回顾了 Hedgehog 信号在肾脏形态发生过程中的现有知识,并强调了我们理解中的差距。此外,我们提出了 Hedgehog 信号在正常和异常肾脏发育中的作用机制。