SLC25A26 exchanges cytosolic AdoMet for mitochondrial AdoHcy

SLC25A26 (S-adenosylmethionine mitochondrial carrier protein) associated with the inner mitochondrial membrane mediates the exchange of cytosolic AdoMet (S-adenosylmethionine) for mitochondrial AdoHcy (S-adenosylhomocysteine). The substrate specificity and countertransport function of SLC25A26 were established from studies of liposomes reconstituted with purified protein in vitro (Agrimi et al. 2004). SLC25A26 mutations that disrupt transport activity have been identified in patients with defects of mitochondrial function, consistent with a requirement for AdoMet uptake from the cytosol to support mitochondrial methylation reactions (Kishita et al. 2015).

SLC25A26（S-腺苷甲硫氨酸线粒体载体蛋白）与线粒体内膜相关联，介导细胞质中的腺苷甲硫氨酸（S-腺苷甲硫氨酸）与线粒体中的腺苷高半胱氨酸（S-腺苷高半胱氨酸）之间的交换。SLC25A26的底物特异性和逆向转运功能通过体外复制的由纯化蛋白组成的脂质体的研究得以确立（Agrimi 等人，2004年）。在具有线粒体功能障碍的患者中，已鉴定出破坏转运活性的SLC25A26突变，这与从细胞质摄取腺苷甲硫氨酸以支持线粒体甲基化反应的需求相一致（Kishita 等人，2015年）。