Dyskerin also known as NAP57 plays a crucial role in the biogenesis of ribonucleoprotein complexes, particularly in the modification of uridine to pseudouridine, which is essential for the proper functioning of ribosomal RNA and, consequently, protein synthesis. This modification enhances the stability and functionality of RNA molecules, impacting cellular processes such as translation and ribosome assembly. Dyskerin forms a small ribonucleoprotein particle with other proteins, including Nopp140, GAR1, NHP2, and Nop10, which localize to the dense fibrillar component of the nucleolus and nuclear Cajal bodies, highlighting dyskerin′s importance in nucleolar function and cellular homeostasis.