From and to the Golgi – defining the Wilson disease protein road map

FEBS Letters

article

Roman S. Polishchuk¹  Elena V. Polishchuk¹ 

[1] Telethon Institute of Genetics and Medicine (TIGEM);ITMO University

关键词: ATP7B;    copper homeostasis;    Golgi apparatus;    membrane trafficking;    Wilson disease;   

DOI  :  10.1002/1873-3468.13575

来源: John Wiley & Sons Ltd.

【 摘 要 】

Recent studies highlight the continued growth in the identification of a variety of cellular functions that involve the Golgi apparatus. Apart from well-known membrane sorting/trafficking and glycosylation machineries, the Golgi harbors molecular platforms operating in intracellular signaling, cytoskeleton organization, and protein quality control mechanisms. One of new emerging Golgi functions consists in the regulation of copper homeostasis by coordinating the relocation and activity of copper transporters. Of these, the Cutransporting ATPase ATP7B (known as Wilson disease protein) plays a key role in the maintenance of the Cu balance in the body via the supply of essential Cu to the systemic circulation and via elimination of excess Cu into the bile. These activities require tightly regulated shuttling of ATP7B between the Golgi and different post-Golgi compartments. Despite significant progress over recent years, a number of issues regarding ATP7B trafficking remain to be clarified. This review summarizes current views on ATP7B trafficking pathways from and to the Golgi and underscores the challenges that should be addressed to define the ATP7B trafficking routes and mechanisms in health and disease.

【 授权许可】

Unknown