Besides, the impairment of stress response by mutations in EIF2AK2 –which encodes a eiF2α kinase–and eiF2B genes–EIF2B1 and EIF2B2– leads to leukoencephalopathy (Leegwater et al., 2001; Mao et al., 2020), hereby suggesting the importance of the UPR in CNS structure. Here, EIF2B2 is linked to Leukoencephalopathy.