Department of Emergency Medicine, University of Pennsylvania, Philadelphia, PA, USA; Center for Resuscitation Science, University of Pennsylvania, Philadelphia, PA, USA.
Neurobiol Dis. 2013 Dec;60:61-79. doi: 10.1016/j.nbd.2013.08.010. Epub 2013 Aug 19.
Axonal injury and degeneration, whether primary or secondary, contribute to the morbidity and mortality seen in many acquired and inherited central nervous system (CNS) and peripheral nervous system (PNS) disorders, such as traumatic brain injury, spinal cord injury, cerebral ischemia, neurodegenerative diseases, and peripheral neuropathies. The calpain family of proteases has been mechanistically linked to the dysfunction and degeneration of axons. While the direct mechanisms by which transection, mechanical strain, ischemia, or complement activation trigger intra-axonal calpain activity are likely different, the downstream effects of unregulated calpain activity may be similar in seemingly disparate diseases. In this review, a brief examination of axonal structure is followed by a focused overview of the calpain family. Finally, the mechanisms by which calpains may disrupt the axonal cytoskeleton, transport, and specialized domains (axon initial segment, nodes, and terminals) are discussed.
轴突损伤和变性,无论是原发性还是继发性,都会导致许多获得性和遗传性中枢神经系统(CNS)和周围神经系统(PNS)疾病的发病率和死亡率,如创伤性脑损伤、脊髓损伤、脑缺血、神经退行性疾病和周围神经病变。钙蛋白酶蛋白酶家族与轴突功能障碍和退化有机制上的联系。虽然横断、机械应变、缺血或补体激活引发轴内钙蛋白酶活性的直接机制可能不同,但在看似不同的疾病中,不受调节的钙蛋白酶活性的下游效应可能相似。在这篇综述中,简要检查了轴突的结构,然后重点概述了钙蛋白酶家族。最后,讨论了钙蛋白酶可能破坏轴突细胞骨架、运输和特殊区域(轴突起始段、节点和末端)的机制。