Mitochondrial basic amino acids transporter (SLC25A29)

Mitochondrial basic amino acids transporter (SLC25A29) is a nuclear-encoded mitochondrial membrane protein belonging to the solute carrier family 25, also known as the mitochondrial carrier family. It functions primarily as a transporter for basic amino acids—including arginine, lysine, homoarginine, and methylarginine—across the inner mitochondrial membrane, facilitating their import into the mitochondrial matrix for protein synthesis and amino acid degradation. SLC25A29 also transports ornithine and histidine at lower efficiencies and is formally recognized as mitochondrial ornithine transporter 3 (ORNT3). This protein plays a secondary and less physiologically significant role overlapping with other mitochondrial transporters involved in the ornithine degradation pathway and the urea cycle. Mutations or altered expression in SLC25A29 may contribute to variability in clinical presentations of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, a metabolic disorder. Despite past reports, SLC25A29 does not reliably transport carnitine or acylcarnitines. It is considered a putative modifier gene in selected metabolic disorders, but there is little evidence of direct drug targeting, known mechanism-of-action-based drugs, validated biomarkers, or explicit safety risks, as of current literature[1][2][3].

Other names

Solute carrier family 25 member 29C14orf69Mitochondrial ornithine transporter 3 (ORNT3)Mitochondrial carnitine/acylcarnitine carrier protein CACLCarnitine/acylcarnitine translocase-likeFLJ38975CACT-like