SLC4A10 is a sodium-coupled bicarbonate transporter that plays critical roles in pH regulation and neuronal function 1. The protein localizes to inhibitory presynapses in neurons and the basolateral membrane of choroid plexus epithelial cells, where it mediates cellular HCO3- uptake to control intracellular pH 23. SLC4A10 is essential for cerebrospinal fluid production, as knockout mice exhibit dramatically reduced brain ventricle volume 3. The transporter specifically regulates GABAergic neurotransmission by controlling presynaptic pH, which is necessary for proper GABA release, while glutamate release remains unaffected 2. Loss of SLC4A10 function reduces neuronal excitability and modulates short-term synaptic plasticity, providing protection against epileptic seizures 34. Biallelic loss-of-function variants in SLC4A10 cause a recognizable neurodevelopmental disorder characterized by hypotonia, developmental delays, intellectual disability, progressive microcephaly, and characteristic brain abnormalities including slit-like lateral ventricles 25. The disorder commonly includes autistic spectrum features and occasional seizures, reflecting the transporter's crucial role in brain development and GABAergic transmission 2. SLC4A10 variants are also associated with systemic water balance regulation through central nervous system mechanisms 6.