Ragged red fibers and COX-negative fibers were specific for mitochondrial disease, as expected, but had low sensitivity, being detected in only 1 patient each (patient IDs 40 and 81, respectively), both of whom were diagnosed with a genetically confirmed mitochondrial disease (pathogenic variants in MT-ND5 and RMND1, respectively). This evidence concerns the gene MT-ND5 and inborn mitochondrial metabolism disorder.