Detailed investigations of DCA were performed to evaluate its effects on behavior, neurologic and/or muscular activity, mitochondrial function, and lactate metabolism in the C. elegans model of FBXL4 mitochondrial disease, with subsequent validation studies performed in zebrafish exposed to mitochondrial translation inhibition with chloramphenicol (CAP) and human fibroblasts from individuals with FBXL4–/– disease. This evidence concerns the gene FBXL4 and mitochondrial disease.