Contrastingly, the ASPA knockout mouse model (CD KO), first constructed in a C57BL/6 background (Matalon et al., 2000) and, due to genetic instability, was later crossed into a SV129/Ev background (Ahmed and Gao, 2013; Ahmed et al., 2016; Gessler et al., 2017), presents with the severe form of Canavan disease, manifesting acute disease progression, such as reduced motor function, spasticity, and lethality as early as 4 weeks of age. Here, ASPA is linked to Canavan disease.