In humans, ITM2B gene mutations lead to two types of autosomal dominant dementia, namely, familial British dementia and familial Danish dementia, characterized by progressive dementia, cerebellar ataxia, and other clinical symptoms.[15, 18, 19] Although the broad expression of ITM2B and its cleavage in tissues have been demonstrated, its physiological functions, particularly in cancer development, have been ignored and need further investigation. Here, ITM2B is linked to cerebellar ataxia.