SZT2 (Seizure Threshold 2) is a large transmembrane protein rich in leucine-rich repeats (LRRs) and primarily localized to the lysosomal membrane. Its core molecular function is to serve as a central scaffold protein of the KICSTOR complex. Under amino acid starvation conditions, it anchors the negative regulator GATOR1 to the lysosomal surface through its scaffolding role, thereby inhibiting excessive activation of the mTORC1 signaling pathway and maintaining cellular growth and metabolic balance. Mutations in the SZT2 gene cause neurodevelopmental disorders such as early-onset epileptic encephalopathy, developmental delay, intellectual disability, and macrocephaly, highlighting its critical role in regulating neuronal excitability. Furthermore, SZT2 is involved in autophagy regulation and the maintenance of lysosomal function; its deficiency leads to impaired clearance of materials within neurons. In neuroscience research, anti-SZT2 antibodies are widely used for exploring epilepsy mechanisms, studying the mTOR signaling pathway, and analyzing neurodevelopmental disease models.