Autosomal dominant cerebellar ataxia, deafness, and narcolepsy (ADCADN) is characterized by late onset cerebellar ataxia, sensory neuronal deafness, narcolepsy-cataplexy and dementia. It has been reported that mutations in DNMT1 cause this disease. DNMT1 is a widely expressed DNA methyltransferase maintaining methylation patterns in development, and mediating transcriptional repression by direct binding to HDAC2.