Feany Mel B, La Spada Albert R
Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA.
Neuron. 2003 Sep 25;40(1):1-2. doi: 10.1016/s0896-6273(03)00600-7.
In this issue of Neuron, two papers provide evidence that polyglutamine (polyQ) proteins disrupt axonal transport. Gunawardena et al. show that normal levels of huntingtin are required for proper axonal transport in Drosophila. In addition, expression of expanded polyQ proteins disrupts axonal transport in larval neurons. Szebenyi and colleagues find that polyQ proteins directly inhibit fast axonal transport using axoplasm from the squid giant axon and suggest that axonal transport defects may be a common feature of polyQ disease pathogenesis.
在本期《神经元》杂志中，两篇论文提供了证据，表明聚谷氨酰胺（polyQ）蛋白会破坏轴突运输。古纳瓦德纳等人表明，正常水平的亨廷顿蛋白对果蝇的轴突正常运输是必需的。此外，扩展的聚谷氨酰胺蛋白的表达会破坏幼虫神经元中的轴突运输。塞贝尼伊及其同事利用乌贼巨轴突的轴浆发现，聚谷氨酰胺蛋白会直接抑制快速轴突运输，并提出轴突运输缺陷可能是聚谷氨酰胺疾病发病机制的一个共同特征。
