Ribosome biogenesis is a ubiquitous and essential process in cells.Disruptions in ribosome biogenesis are deleterious to development and underlie a spectrum of diseases known collectively as ribosomopathies,such as Shwachman-Bodian-Diamond syndrome (SBDS).The main characteristics of SBDS are exocrine pancreatic dysfunction,hematologic abnormalities and skeletal defects.