COQ2 and Nephropathy: 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.