This gene encodes a ribonuclease (RNase) III double-stranded RNA-specific ribonuclease and subunit of the microprocessor protein complex, which catalyzes the initial processing step of microRNA (miRNA) synthesis. The encoded protein cleaves the stem loop structure from the primary microRNA (pri-miRNA) in the nucleus, yielding the precursor miRNA (pre-miRNA), which is then exported to the cytoplasm for further processing. In a human cell line lacking a functional copy of this gene, canonical miRNA synthesis is reduced. Somatic mutations in this gene have been observed in human patients with kidney cancer. DROSHA (Drosha Ribonuclease III) is a Protein Coding gene. Diseases associated with DROSHA include Combat Disorder and Murray Valley Encephalitis. Among its related pathways are mRNA Capping and Gene Expression. GO annotations related to this gene include poly(A) RNA binding and RNA binding.