ENPP1 and hyperinsulinemic hypoglycemia, familial, 4: Preclinical studies of INZ-701 in Enpp1asj mice dosed between 0.2 and 5 mg/kg every other day restored circulating levels of PPi and prevented pathological calcification in all the tested organs, restored growth parameters, corrected bone defects, improved clinical signs, and decreased mortality, demonstrating the potential of INZ-701 to treat ENPP1 deficiency in humans (115).