As a model of mitochondrial diseases, we used transmitochondrial cybrids (Bacman et al, 2020; Hashimoto et al, 2015) harboring wild-type mtDNA and two disease-causing mutations: m.13513 G > A (MT-ND5) (Shanske et al, 2008; Wang et al, 2010, 2023) and m.8344 A > G (MT-tRNA-Lys) (Silvestri et al, 1993; Shoffner et al, 1990). This evidence concerns the gene MT-ND5 and inborn mitochondrial metabolism disorder.