SLC25A18,22 import L-Glu, H+

Mitochondrial glutamate carriers 1 and 2 (SLC25A22, GC1, and SLC25A18, GC2) belong to the mitochondrial carrier family of transport proteins that shuttle substrates, metabolites, and cofactors through the mitochondrial membrane, connecting the cytosol to the mitochondrial matrix. Glutamate (Glu) can be co-transported with H+ via SLC25A18 and SLC25A22, located on the inner mitochondrial membrane (Fiermonte et al., 2002). Defects in SLC25A22 can cause early infantile epileptic encephalopathy 3 (EIEE3; MIM:609304), a severe neonatal epilepsy characterized by very early onset, erratic and fragmentary myoclonus. With no treatment available, children with EIEE3 die within 1 or 2 years of birth or survive in a vegetative state (Molinari et al., 2005; reviewed in Spoto et al., 2021).

线粒体谷氨酸载体1和2（SLC25A22、GC1、SLC25A18及GC2）隶属于线粒体载体家族的转运蛋白，这些蛋白负责将底物、代谢物和辅因子穿梭于线粒体膜，从而将细胞质与线粒体基质相连。谷氨酸（Glu）可通过位于线粒体内膜的SLC25A18和SLC25A22与H+共转运（Fiermonte等，2002年）。SLC25A22的缺陷可导致早期婴儿期癫痫性脑病3型（EIEE3；MIM：609304），这是一种严重的围产期癫痫，以发病早、肌阵挛不规律且碎片化为特征。由于目前尚无治疗方法，患有EIEE3的儿童在出生后1至2年内死亡或在植物状态下存活（Molinari等，2005年；Spoto等，2021年综述）。