CLN3 transports GalCer to plasma membrane

CLN3 (neuronal ceroid-lipofuscinosis 3) protein (battenin), which has five transmembrane domains, including a GalCer lipid raft-binding domain, is responsible for the transport of GalCer from ER/Golgi to lipid rafts in the membrane. The details of this process need to be clarified (Rusyn et al., 2008; reviewed by Liu & Li, 2013). CLN3 transport of proteins and glycolipids to the plasma membrane and lysosomes is part of various processes. Deficiencies in CLN3 are the cause of neuronal ceroid-lipofuscinosis 3 (CLN3, "Batten's disease," MIM:204200; reviewed by Cotman & Staropoli, 2012; Mirza et al., 2019).

CLN3（神经元蜡样质脂褐素沉积症3型蛋白，亦称battenin）具有五个跨膜结构域，其中包括一个GalCer脂筏结合域，负责将GalCer从内质网/高尔基体运输至细胞膜上的脂筏。此运输过程之细节亟待阐明（Rusyn等，2008；经Liu与Li，2013年综述）。CLN3在将蛋白和糖脂运输至质膜和溶酶体的过程中扮演着多种角色的部分。CLN3的缺乏是导致神经元蜡样质脂褐素沉积症3型（CLN3，即Batten病，MIM：204200；经Cotman与Staropoli，2012年；Mirza等，2019年综述）的病因。