AP-3-dependent trafficking and disease: the first decade
AP-3-dependent trafficking and disease: the first decade
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DOI：
10.1016/j.ceb.2009.04.014
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发表时间：
2009-08-01
影响因子：
7.5
通讯作者：
Dell'Angelica, Esteban C.
中科院分区：
文献类型：
--
作者：
Dell'Angelica, Esteban C.
在大多数真核细胞中，衔接蛋白（AP）-3复合物定义了膜相关蛋白在细胞内运输的途径。十年前，AP-3的遗传缺陷与一种名为Hermansky-Pudlak综合征的人类孟德尔疾病有关，其特征是溶酶体相关细胞器（如黑素体和血小板致密颗粒）的生物发生和功能异常。近年来，对这一运输途径的研究已显着扩大了其视野，包括进化上不同的真核模型，并拥抱功能基因组学和蛋白质组学方法。这些研究使人们对这一途径在内体-溶酶体系统中蛋白质运输和细胞器生物合成中的具体作用有了新的认识。
The adaptor protein (AP)-3 complex defines a pathway for the intracellular trafficking of membrane-associated proteins in most eukaryotic cells. Ten years ago, genetic defects in AP-3 were linked to a human Mendelian disease, named Hermansky-Pudlak syndrome, characterized by abnormal biogenesis and function of lysosome-related organelles such as melanosomes and platelet dense granules. During recent years, research on this trafficking pathway has significantly expanded its horizons to include evolutionarily divergent eukaryotic models and to embrace functional genomics and proteomics approaches. These studies have brought into focus ideas about the specific roles of this pathway in protein trafficking and organelle biogenesis within the endosomal-lysosomal system.