ADCK4 disease typically manifests as an isolated nephropathy with occasional extrarenal symptomatology on contrast to the mutations in PDSS2, COQ2, and COQ6, those renal symptoms usually occur as part of a multisystemic disease complex encompassing progressive encephalopathy, seizures, and hypertrophic cardiomyopathy.[13] In patients with ADCK4 mutant, advanced chronic kidney disease (CKD) at time of diagnosis was more prevalent than in patients with NPHS2 mutation. Here, COQ8B is linked to glycogen storage disease VI.