SBDS and Shwachman-Diamond syndrome: Underscoring the importance of this step of ribosome maturation, deficiency of SBDS or EFL1 causes Shwachman-Diamond Syndrome (SDS), a rare inherited ribosomopathy characterized by bone marrow failure, pancreatic insufficiency, skeletal abnormalities, and predisposition to hematopoietic malignancies25–27.