In patients with mutations in coenzyme Q2 (COQ2), COQ6, coenzyme Q8B (ADCK4), or decaprenyl diphosphate synthase subunit 2 (PDSS2), genes involved in CoQ10 biosynthesis, early initiation of CoQ10 supplementation may be beneficial to reduce proteinuria and FSGS progression [38–41]. Here, PDSS2 is linked to focal segmental glomerulosclerosis.