Failure to resolve DNA single strand breaks (SSB) is also associated with a number of cerebellar atrophies [13] and these include ataxia oculomotor apraxia type 1 (AOA1) and spinocerebellar ataxia with axonal neuropathy (SCAN1). Here, TDP1 is linked to spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 1.