Department of Neurobiology, University of Massachusetts Medical School, Worcester, MA 01605, USA.
Science. 2012 Jul 27;337(6093):481-4. doi: 10.1126/science.1223899. Epub 2012 Jun 7.
Axonal and synaptic degeneration is a hallmark of peripheral neuropathy, brain injury, and neurodegenerative disease. Axonal degeneration has been proposed to be mediated by an active autodestruction program, akin to apoptotic cell death; however, loss-of-function mutations capable of potently blocking axon self-destruction have not been described. Here, we show that loss of the Drosophila Toll receptor adaptor dSarm (sterile α/Armadillo/Toll-Interleukin receptor homology domain protein) cell-autonomously suppresses Wallerian degeneration for weeks after axotomy. Severed mouse Sarm1 null axons exhibit remarkable long-term survival both in vivo and in vitro, indicating that Sarm1 prodegenerative signaling is conserved in mammals. Our results provide direct evidence that axons actively promote their own destruction after injury and identify dSarm/Sarm1 as a member of an ancient axon death signaling pathway.
轴突和突触退化是周围神经病变、脑损伤和神经退行性疾病的标志。轴突退化被认为是由主动的自毁程序介导的,类似于细胞凋亡;然而,还没有描述能够强烈阻断轴突自毁的功能丧失突变。在这里,我们表明果蝇 Toll 受体接头 dSarm(无菌 α/Armadillo/Toll-白细胞介素受体同源结构域蛋白)的缺失在轴突切断后数周内自主抑制了瓦勒变性。切断的小鼠 Sarm1 缺失轴突在体内和体外都表现出显著的长期存活,表明 Sarm1 促退化信号在哺乳动物中是保守的。我们的结果提供了直接的证据,表明轴突在受伤后主动促进自身的破坏,并确定 dSarm/Sarm1 是古老的轴突死亡信号通路的一个成员。