ACSBG1 and glycogen storage disease VI: In contrast, μMT−/− BG1 mice were found to be highly susceptible to spontaneous CNS disease, with ~80% of μMT−/− BG1 mice developing chronic clinical disease, a fundamentally distinct disease course as compared to the relapsing-remitting disease most often observed in WT BG1 and Rag−/− BG1 mice (Sasaki et al., 2014).