TMEFF1 and Sandhoff disease: However, tomoregulin-1 is upregulated in a feline model of GM2 gangliosidosis, which is an authentic biochemical and morphological replica of human Sandhoff disease (Cork et al., 1977), and tomoregulin-1 shows a shift in its intracellular location to the nucleus and seems to function in primary dendrite initiation (Siegel et al., 2002).