DROSHA and hereditary hemorrhagic telangiectasia: Several disease-associated alleles of Drosha have been identified in humans, including missense mutations in the RNase III domains of Drosha in patients with Wilms tumor10,11,12,13,14,15 and missense mutations in the P-rich and the RS-rich regions of Drosha (e.g., P100L and R279L) in patients with hereditary hemorrhagic telangiectasia (HHT).16