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文章简介人类自噬基因EPG5突变会导致多重系统紊乱Vici综合征。这篇论文证明了EPG5作为Rab7的效应蛋白,特异性介导自噬小体与晚期内体/溶酶体之间的融合。EPG5可以与Rab7及晚期内体/溶酶体上的R-SNARE蛋白VAMP7/8直接作用,进而定位于晚期内体/溶酶体。EPG5也可以与自噬蛋白LC3/LGG-1(人类和线虫中Atg8的同源基因),及预组装的Qabc-SNARE复合物STX17-SNAP29结合,从而通过EPG5将自噬小体定
INTRODUCTION Mutations in human autophagy gene EPG5 lead to multiple system disorders in Vici syndrome. This paper demonstrates that EPG5 acts as a Rab7 effector protein that specifically mediates the fusion between autophagosomes and late endosome / lysosomes. EPG5 can directly interact with Rab7 and the late endosomal / lysosomal R-SNARE protein VAMP7 / 8, targeting the late endosome / lysosome. EPG5 can also bind to the autophagy protein LC3 / LGG-1 (homologue of Atg8 in humans and nematodes), as well as to the preassembled Qabc-SNARE complex STX17-SNAP29, so that autophagosomes can be targeted by EPG5