For example, mutations in OSGEP, TP53RK, TPRKB, LAGE3 and C14orf142, encoding all five subunits of the human cytoplasmic KEOPS complex, lead to defective t6A modification and Galloway-Mowat syndrome (GAMOS), characterised by early-onset nephrotic syndrome, microcephaly and brain anomalies (35). Here, OSGEP is linked to Galloway-Mowat syndrome.