The calcium-binding mitochondrial carrier protein (SLC25A12), also named as ARALAR or AGC1, is an aspartate-glutamate exchange protein responsible for transporting mitochondrial aspartate across the mitochondrial inner membrane in exchange for cytosolic glutamate. SLC25A12 and other proteins of the aspartate-glutamate carrier (AGC) group are required for the transfer of mitochondrial aspartate to the cytosol, a key step in urea synthesis. Research studies using SLC25A12-knockout mice indicate that SLC25A12 plays an important role in proper CNS myelination. Mice lacking SLC25A12 suffer from hypomyelination as a result of a lack of oligodendrocyte maturation caused by decreased brain N-acetylaspartate levels. Mutation of the corresponding SLC25A12 gene can result in global cerebral hypomyelination and severe psychomotor retardation, caused by deficient SLC25A12 activity and limited mitochondrial aspartate efflux.
