Loss-of-function mutations in OSGEP, TP53RK, TPRKN or LAGE3, which encode the four subunits of the KEOPS complex (Kinase, Endopeptidase and Other Protein of small Size) result in Galloway-Mowat syndrome (GAMOS, #MIM251300), an early-onset nephrotic disorder with severe PM [82]. The gene discussed is OSGEP; the disease is Galloway-Mowat syndrome.