Mutation Gene name % affected tumors Hotspot residues Mechanism of action Location
GNAS G protein Subunit Alpha S 4.45% R201 Q227 Reduce rate of GTP hydrolysis of active bound Gα, resulting in continuous signaling of Gαs. testis, small intestine, pituitary, bile tract
GNAQ G Protein Subunit Alpha Q 3.36% Q209 R183 Activate GEF, Trio and Rho GTPase signaling, activating MAPK-pathway. eye, meninges
GNA11 G Protein Subunit Alpha 11 2.49%