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首页> 外文期刊>Journal of Cell Science >CORVET, CHEVI and HOPS - multisubunit tethers of the endo-lysosomal system in health and disease
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CORVET, CHEVI and HOPS - multisubunit tethers of the endo-lysosomal system in health and disease

机译：Corvet，Chevi和HOPS - 健康和疾病中尾溶酶体系统的多相管

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摘要

Multisubunit tethering complexes (MTCs) are multitasking hubs that form a link between membrane fusion, organelle motility and signaling. CORVET, CHEVI and HOPS are MTCs of the endo-lysosomal system. They regulate the major membrane flows required for endocytosis, lysosome biogenesis, autophagy and phagocytosis. In addition, individual subunits control complex-independent transport of specific cargoes and exert functions beyond tethering, such as attachment to microtubules and SNARE activation. Mutations in CHEVI subunits lead to arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome, while defects in CORVET and, particularly, HOPS are associated with neurodegeneration, pigmentation disorders, liver malfunction and various forms of cancer. Diseases and phenotypes, however, vary per affected subunit and a concise overview of MTC protein function and associated human pathologies is currently lacking. Here, we provide an integrated overview on the cellular functions and pathological defects associated with CORVET, CHEVI or HOPS proteins, both with regard to their complexes and as individual subunits. The combination of these data provides novel insights into how mutations in endo-lysosomal proteins lead to human pathologies.
机译：多相管束缚复合物（MTCS）是多任务枢纽，其形成膜融合，细胞器运动和信号传导之间的联系。 Corvet，Chevi和HOPS是内泌体系统的MTC。它们调节内吞作用，溶酶体生物发生，自噬和吞噬作用所需的主要膜流量。此外，单个亚基控制特定货物的复杂传输，并施加超出束缚的函数，例如对微管和圈套激活的附着。 Chevi亚基的突变导致腺血清症，肾功能紊乱和胆汁抑制（ARC）综合征，而猪的缺陷，特别是啤酒花，啤酒花与神经变性，色素沉着病症，肝脏发生故障和各种形式的癌症有关。然而，目前缺乏疾病和表型，每次受影响的亚基各种亚基和简要概述MTC蛋白质功能和相关人类病理的概述。在这里，我们提供了与蜂窝功能的综合概述，以及与Corvet，Chevi或啤酒花蛋白相关的病理缺陷，两者都是关于它们的复合物和单个亚基。这些数据的组合提供了对内泌体蛋白质中突变导致人类病理的突变的新颖见解。

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