As reviewed by Chandran et al. (2007) [147] and Cai et al. (2008) [148], to date four ALS2-deficient mouse models have been developed [149,150,151,152] to shed light on the impact of alsin deficiency in ALS, but they largely failed to recapitulate hallmarks of motor neuron disease [149,150,151,153]. The gene discussed is ALS2; the disease is motor neuron disorder.