The IRP1 gene (ACO1) has been associated with cutaneous malignant melanoma (Yang et al., 2010) and neuropathic pain in HIV-infected patients (Kallianpur et al., 2014), while the IRE-binding activity of IRP1 was reported to be increased in Friedreichs' ataxia (Lobmayr et al., 2005) and in Parkinson's disease (Faucheux et al., 2002). Here, ACO1 is linked to Friedreich ataxia.