The commonest genes associated with adult-onset FSGS (COL4A3, COL4A4, COL4A5) have ocular abnormalities as does GLA (Fabry disease), but not ACTN4, CD2AP, INF2 or TRPC6. LMX1B and MYH9 are infrequent causes of FSGS but ocular features are common. Here, ACTN4 is linked to Fabry disease.