Abstract
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 language | English (US) |
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Pages (from-to) | 1483-1494 |
Number of pages | 12 |
Journal | RNA biology |
Volume | 11 |
Issue number | 12 |
DOIs | |
State | Published - Dec 1 2014 |
Keywords
- 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