As a component of the GATOR2 complex, functions as an activator of the amino acid-sensing branch of the mTORC1 signaling pathway (  ). The GATOR2 complex indirectly activates mTORC1 through the inhibition of the GATOR1 subcomplex (  ). GATOR2 probably acts as an E3 ubiquitin-protein ligase toward GATOR1 . In the presence of abundant amino acids, the GATOR2 complex mediates ubiquitination of the NPRL2 core component of the GATOR1 complex, leading to GATOR1 inactivation . In the absence of amino acids, GATOR2 is inhibited, activating the GATOR1 complex ( , ). Within the GATOR2 complex, MIOS is required to prevent autoubiquitination of WDR24, the catalytic subunit of the complex . The GATOR2 complex is required for brain myelination (By similarity).
Subcellular locations: Lysosome membrane