RNA-guided isomerization of uridine to pseudouridine - Pseudouridylation

Yi Tao Yu, U. Thomas Meier

Research output: Contribution to journalReview articlepeer-review

86 Scopus citations


Box H/ACA ribonucleoproteins (RNPs), each consisting of one unique guide RNA and 4 common core proteins, constitute a family of complex enzymes that catalyze, in an RNA-guided manner, the isomerization of uridines to pseudouridines (Ψs) in RNAs, a reaction known as pseudouridylation. Over the years, box H/ACA RNPs have been extensively studied revealing many important aspects of these RNA modifying machines. In this review, we focus on the composition, structure, and biogenesis of H/ACA RNPs. We explain the mechanism of how this enzyme family recognizes and specifies its target uridine in a substrate RNA. We discuss the substrates of box H/ACA RNPs, focusing on rRNA (rRNA) and spliceosomal small nuclear RNA (snRNA). We describe the modification product Ψ and its contribution to RNA function. Finally, we consider possible mechanisms of the bone marrow failure syndrome dyskeratosis congenita and of prostate and other cancers linked tomutations in H/ACA RNPs.

Original languageEnglish (US)
Pages (from-to)1483-1494
Number of pages12
JournalRNA biology
Issue number12
StatePublished - Dec 1 2014


  • Dyskeratosis congenita
  • H/ACA
  • Prostate cancer
  • Pseudouridine
  • RNA-guided
  • RRNA
  • Ribonucleoproteins
  • Rna modification
  • SnoRNA
  • Spliceosomal small nuclear rna

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology


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