Braun et al. [1] found that the subunits coded by the four genes LAGE3/OSGEP/TP53RK/TPRKB constitute a highly conserved kinase-endopeptidase and other proteins of small size complex (KEOPS), which is one of the key factors in the pathogenesis of GAMOS. Here, LAGE3 is linked to Galloway-Mowat syndrome.