HSPA5 and GM1 gangliosidosis: In a mouse model with GM1-gangliosidosis, the upregulation of GRP78/BiP and CHOP and the activation of JNK2 and caspase-12 can induce neuronal cell death, suggesting that the UPR can induce the accumulation of the sialoglylipid GM1 in the neuronal cells and then cause neuronal apoptosis [102].