MTFMT and inborn mitochondrial metabolism disorder: Studies using a Mtfmt knockout mouse fibroblast model lacking exon 4 in MTFMT demonstrated that MTFMT is not an absolute requirement for initiation of translation and elongation of mitochondrial protein synthesis in mice but deficiency of this protein results in reduced efficiency of this process and downstream oxidative phosphorylation defects.24 This may contribute to the overall milder disease course observed in the MTFMT‐related mitochondrial disease presented here compared with typical LS.