SLC25A13 encodes citrin, a mitochondrial electrogenic aspartate/glutamate antiporter that mediates the malate-aspartate shuttle, a critical NADH-shuttle pathway in hepatocytes 1. The transporter catalyzes consecutive, stepwise substrate exchange, promoting aspartate efflux while facilitating glutamate and proton entry into mitochondria 2. SLC25A13 also transports L-cysteinesulfinate in exchange for glutamate and proton, or aspartate without proton translocation 3. Notably, it lacks transport activity for gamma-aminobutyric acid 4. Purinosome assembly occurs proximally to SLC25A13 at the mitochondrial membrane, enabling uptake of glycine, aspartate, and glutamate essential for purine synthesis 5. Biallelic SLC25A13 mutations cause citrin deficiency (CD), a recessive liver disease with age-dependent phenotypes 6. CD impairs hepatic metabolic pathways including glycolysis, gluconeogenesis, lipogenesis, and the urea cycle, resulting in energy deficit in hepatocytes 6. Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) represents the major pediatric phenotype 7. SLC25A13 mutations demonstrate significant geographic distribution variation, with northern Chinese populations showing greater allelic heterogeneity 7. Current management includes dietary intervention with medium-chain triglycerides as first-line treatment, with liver transplantation remaining the sole curative option for severe cases 6.