Department of Nephrology and Hypertension, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Nephrology and Hypertension, University Medical Center Utrecht, Utrecht, The Netherlands.
Trends Cell Biol. 2015 Jun;25(6):317-9. doi: 10.1016/j.tcb.2015.03.005. Epub 2015 Apr 27.
Juvenile renal failure is commonly caused by the ciliopathy nephronophthisis (NPHP). Since all NPHP genes regulate cilia function, it has been assumed that NPHP onset is due to cilia loss. However, recent data suggest that DNA damage caused by replication stress, possibly concomitant with or upstream of cilia dysfunction, causes NPHP.
儿童肾衰竭通常由纤毛病肾单位肾痨(NPHP)引起。由于所有 NPHP 基因都调节纤毛功能,因此人们一直认为 NPHP 的发生是由于纤毛丢失所致。然而,最近的数据表明,由复制应激引起的 DNA 损伤,可能与纤毛功能障碍同时发生或在其之前,导致 NPHP。