For example, mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS) and a select few defects in nuclear encoded genes (CoQ2, CoQ6, PDSS2, and ADCK4) that impair coenzyme Q10 biosynthesis, classically may present with steroid‐resistant nephrotic syndrome and focal segmental glomerular sclerosis (FSGS) with evidence of podocyte effacement, dysmorphic mitochondria, and arteriolar hyalinosis (Ashraf et al., 2013; Gucer et al., 2005; Seidowsky et al., 2013; Yanagihara, Oyama, Tanaka, Nakaji, & Nishimura, 2001). Here, COQ6 is linked to focal segmental glomerulosclerosis.