CAJ | 학술논문

随着高等生物中十几个新的参与囊泡运输的Hermansky-Pudlak综合征(HPS)蛋白质的发现,认为可能存在一类新的囊泡运输通路.该通路主要由新近鉴定的3个被称为溶酶体相关细胞器生物发生复合体(BLOC)所组成,被分别命名为BLOC-1、BLOC-2和BLOC-3.越来越多的证据表明这些复合体与以前认识较清楚的AP3和HOPS复合体共同在胞内体运输中起重要作用.这些复合体之间的相互作用构成了以胞内体和细胞骨架为连接纽带的参与蛋白质运输的复杂网络.该网络中的每个节点的相互作用可区分为复合体内和复合体外相互作用两大类.复合体之间的联系可以是来自不同复合体亚基间的直接相互作用,也可以通过耦联的节点联结不同的复合体.解析这一复杂网络有助于进一步了解参与蛋白质和膜运输这一动态而精细网络的结构与功能.一旦该网络结构得到破坏,则可能导致如HPS这类囊泡运输或细胞器发生障碍性疾病.
수착고등생물중십궤개신적삼여낭포운수적Hermansky-Pudlak종합정(HPS)단백질적발현,인위가능존재일류신적낭포운수통로.해통로주요유신근감정적3개피칭위용매체상관세포기생물발생복합체(BLOC)소조성,피분별명명위BLOC-1、BLOC-2화BLOC-3.월래월다적증거표명저사복합체여이전인식교청초적AP3화HOPS복합체공동재포내체운수중기중요작용.저사복합체지간적상호작용구성료이포내체화세포골가위련접뉴대적삼여단백질운수적복잡망락.해망락중적매개절점적상호작용가구분위복합체내화복합체외상호작용량대류.복합체지간적련계가이시래자불동복합체아기간적직접상호작용,야가이통과우련적절점련결불동적복합체.해석저일복잡망락유조우진일보료해삼여단백질화막운수저일동태이정세망락적결구여공능.일단해망락결구득도파배,칙가능도치여HPS저류낭포운수혹세포기발생장애성질병.
With the identification of more than a dozen novel Hermansky-Pudlak Syndrome (HPS) proteins in vesicle trafficking in higher eukaryotes, a new class of trafficking pathways has been described. It mainly consists of three newly-defined protein complexes, BLOC-1, -2, and -3. Compelling evidence indicates that these complexes together with two other well-known complexes,AP3 and HOPS, play important roles in endosomal transport. The interactions between these complexes form a network in protein trafficking via endosomes and cytoskeleton. Each node of this network has intra-complex and extra-complex interactions. These complexes are connected by direct interactions between the subunits from different complexes or by indirect interactions through coupling nodes that interact with two or more subunits from different complexes. The dissection of this network facilitates the understanding of a dynamic but elaborate transport machinery in protein/membrane trafficking. The disruption of this network may lead to abnormal trafficking or defective organellar development as described in patients with Hermansky-Pudlak syndrome.