GON7 and Galloway-Mowat syndrome: The exposure of this hydrophobic surface due to the absence of GON7 in the GAMOS patients may affect the solubility and activity of the KEOPS complex, and indeed, our data from experiments on cell lines further indicate that GON7 contributes to the stability of the KEOPS complex and/or to the maintenance of the correct (catalytically active) quaternary structure as evidenced by the decrease in KEOPS subunits protein levels observed in GON7 patient cells.