As DROSHA is the core enzyme for miRNA biogenesis and miRNA dysregulation has been implicated in various neurological diseases including Alzheimer’s disease (AD) and ALS8,60,61, the mechanisms regulating Drosha expression including splicing, post-translational modification, and degradation have been intensively studied19–23,44. Here, DROSHA is linked to early-onset autosomal dominant Alzheimer disease.