Noncoding RNAs of the H/ACA family.

The H/ACA RNAs are an abundant family of trans-acting, noncoding RNAs found in eukaryotes and archaea. More than 100 H/ACA RNAs are known to exist in humans. The function of the majority of the identified H/ACA RNAs is to guide sites-pecific pseudouridylation of ribosomal RNA. In eukaryotes, H/ACA RNAs also mediate the processing of pre-rRNA, provide the template for telomere synthesis, and guide pseudouridylation of other classes of target RNAs (e.g., small nuclear RNAs [snRNAs]). Thus, currently, the H/ACA RNAs are known to be integrally involved in the production of both ribosomes and spliceosomes, and in the maintenance of chromosome integrity. In addition, dozens of H/ACA RNAs have been identified for which no function has yet been determined. The H/ACA RNAs select and present substrate molecules via base pairing. All H/ACA RNAs contain conserved sequence elements (box H and box ACA) and assemble with a core set of four proteins to form functional ribonucleoprotein complexes (RNPs). Mutations in key RNA and protein components of H/ACA RNPs result in dyskeratosis congenita, a serious multisystem genetic disease. Impressive progress has been made very recently in understanding the biogenesis, trafficking, and function of H/ACA RNPs.