2.A.29.2.1
Oxoglutarate/malate antiporter, OMA, or malate/aspartate shuttle, MAS. Also transports porphyrin derivatives: Fe-protoporphyrin IX, coproporphyrin III, hemin, etc. (Kabe et al., 2006). Plays roles in the malate-aspartate shuttle, (MAS), the oxoglutarate-isocitrate shuttle, OIS, and gluconeogenesis.  Functional residues have been identified (Cappello et al. 2007).  The MAS plays a pivotal role in transporting cytosolic reducing equivalents - electrons - into the mitochondria for energy conversion at the electron transport chain (ETC) and in the process of oxidative phosphorylation (Koch et al. 2024).  Inherited bi-allelic pathogenic variants in five of the seven components of the MAS have been described and cause a wide spectrum of symptoms including early-onset epileptic encephalopathy (Koch et al. 2024).