Figure 1

OCT, lactic acid and skeletal muscle results. (A,B) Panel A and Panel B show, respectively, average and single quadrant RNFL thickness in the tree groups (Controls, DOA, WS). ***p < 0. 001 one-way ANOVA test. (C) Panel C shows sectorial GCL thickness.RNFL: retinal nerve fiber layer; AVG: average; T: temporal; S: superior; N: nasal; I: inferior; int: internal; ext: external; GCL: ganglion cells layer; DOA: dominant optic atrophy; WS: Wolfram syndrome. ***p < 0. 001 one-way ANOVA test. (D) Lactic acid measured in blood at baseline, after exercise and after recovery time in 12 WS patients. Normal values: 5–22 mg/dL. (E) COX/SDH, HE and Gomori modified trichrome staining of the muscle biopsy from patient n. 10. Two COX negative fibers (blue fibers highlighted in the insets), two fibers with central nuclei and one fiber with cytoplasmic bodies are pointed by arrows. Bars: 100 µm.