Department of Molecular and Cellular Physiology, and Howard Hughes Medical Institute, Stanford University, 1050 Arastradero Road, Palo Alto, CA 94304-5543, USA.
Science. 2010 Sep 24;329(5999):1663-7. doi: 10.1126/science.1195227. Epub 2010 Aug 26.
Presynaptic nerve terminals release neurotransmitters repeatedly, often at high frequency, and in relative isolation from neuronal cell bodies. Repeated release requires cycles of soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE)-complex assembly and disassembly, with continuous generation of reactive SNARE-protein intermediates. Although many forms of neurodegeneration initiate presynaptically, only few pathogenic mechanisms are known, and the functions of presynaptic proteins linked to neurodegeneration, such as α-synuclein, remain unclear. Here, we show that maintenance of continuous presynaptic SNARE-complex assembly required a nonclassical chaperone activity mediated by synucleins. Specifically, α-synuclein directly bound to the SNARE-protein synaptobrevin-2/vesicle-associated membrane protein 2 (VAMP2) and promoted SNARE-complex assembly. Moreover, triple-knockout mice lacking synucleins developed age-dependent neurological impairments, exhibited decreased SNARE-complex assembly, and died prematurely. Thus, synucleins may function to sustain normal SNARE-complex assembly in a presynaptic terminal during aging.
突触前神经末梢反复释放神经递质,通常频率较高,且与神经元胞体相对隔离。反复释放需要可溶性 N-乙基马来酰亚胺敏感因子附着蛋白受体(SNARE)复合物的组装和拆卸循环,伴随着反应性 SNARE 蛋白中间产物的持续产生。尽管许多形式的神经退行性变始于突触前,但已知的致病机制很少,与神经退行性变相关的突触前蛋白(如α-突触核蛋白)的功能仍不清楚。在这里,我们表明,持续的突触前 SNARE 复合物组装需要由突触核蛋白介导的非经典伴侣活性来维持。具体而言,α-突触核蛋白直接与 SNARE 蛋白突触融合蛋白 2/囊泡相关膜蛋白 2(VAMP2)结合,并促进 SNARE 复合物的组装。此外,缺乏突触核蛋白的三敲除小鼠表现出年龄依赖性的神经功能障碍,SNARE 复合物的组装减少,并过早死亡。因此,突触核蛋白可能在衰老过程中维持正常的突触前末端 SNARE 复合物组装。