AICA riboside was also tested in vivo in three different mouse models of COX deficiency, in which BZ had also been tested, namely Surf1−/−, a constitutive Sco2 knockout/knockin mouse (Sco2KO/KI), ACTA-Cox15−/− [129]. The gene discussed is COX15; the disease is hyperinsulinemic hypoglycemia, familial, 4.