Strikingly, while the deletion c.813_816del (rs63749877) in the GRN gene in heterozygosis is also associated with FTD, the same mutation when presented in homozygosis causes adult-onset NCL, revealing a remarkable link between neurodegeneration and lipofuscinogenesis (Smith et al., 2012). The gene discussed is GRN; the disease is neuronal ceroid lipofuscinosis.