Remarkably, mNT KO mice exhibit iron accumulation, mitochondrial dysfunction, decreased striatal tyrosine hydroxylase (TH), and dopamine levels and motor deficits, representing many of the characteristics of early neurodegeneration in PD [207], underlining the importance of this pathway in avoiding the hyper activation of IRP1 under oxidative stress. The gene discussed is ACO1; the disease is Parkinson disease.