A missense mutation in the DNA methyltransferase 1 gene (DNMT1) causes the neurodegenerative disease ADCA-DN (Autosomal dominant cerebellar ataxia, deafness and narcolepsy), where three neuronal cell types particularly vulnerable to autoimmunity are lost: the hypocretin neurons (lost in narcolepsy), the cerebellar Purkinje neurons (lost in paraneoplastic cerebellar neurodegeneration), and neurons of the inner ear (lost in autoimmune hearing loss) [27], showing that changes in DNMT1 activity affects post mitotic neurons with devastating consequences. Here, DNMT1 is linked to neurodegenerative disease.