Drosha: a new tumor suppressor in pineoblastoma

Abstract

To investigate the pathogenesis and target the vulnerability of human pineoblastoma, researchers have developed multiple genetically engineered mouse models that represent distinct molecular subtypes of the disease. In this issue of Genes & Development, Fraire and colleagues (doi:10.1101/gad.352485.124) examined the roles of key microRNA (miRNA) processing components Drosha and Dicer1. Loss of either Drosha or Dicer1 partially mimicked the tumorigenic effects of Rb1 deletion by promoting cell cycle progression through the derepression of Plagl2 and cyclin D2. This work reveals a novel mechanism of pineoblastoma development driven by disrupted miRNA processing and highlights a potential therapeutic strategy targeting downstream proliferative drivers.