In cultured cybrid cells harboring various mtDNA mutations and in fibroblasts from patients with mutations of the nuclear gene Sco2, responsible for COX deficiency, Acin-Perez et al. showed that exposure to 1 mM 8-Br-cAMP induced a marked stimulation of COX activity, in parallel with increases in cell respiration and ATP synthesis [127]. Here, COX8A is linked to hyperinsulinemic hypoglycemia, familial, 4.