Currently, premature stop mutation mouse models for other genes are being used to evaluate nonsense suppression therapy, including the Idua-W392X mouse model of human Hurler syndrome (mucopolysaccharidosis type I-H) and a cystic fibrosis transmembrane conductance regulator model (Zhang et al., 2014; Gunn et al., 2014; Wang et al., 2012). Here, CFTR is linked to Hurler syndrome.