Our study discovered that the dysfunction of SLIRP in human HEK293T cells results in impaired mitochondrial respiration due to a deficiency of complex IV and a decrease in the activity and amount of complex I. These findings are consistent with clinical studies that have shown mutations in the SLIRP gene lead to mitochondrial encephalomyopathy accompanied by a decrease in quantities of the same complexes [17]. This evidence concerns the gene SLIRP and mitochondrial encephalomyopathy.