C/D box snoRNAs (SNORDs) are an abundantly expressed class of short, non-coding RNAs that have been long known to perform 2′-O-methylation of rRNAs. However, approximately half of human SNORDs have no predictable rRNA targets, and numerous SNORDs have been associated with diseases that show no defects in rRNAs, among them Prader-Willi syndrome, Duplication 15q syndrome and cancer. This apparent discrepancy has been addressed by recent studies showing that SNORDs can act to regulate pre-mRNA alternative splicing, mRNA abundance, activate enzymes, and be processed into shorter ncRNAs resembling miRNAs and piRNAs. Furthermore, recent biochemical studies have shown that a given SNORD can form both methylating and non-methylating ribonucleoprotein complexes, providing an indication of the likely physical basis for such diverse new functions. Thus, SNORDs are more structurally and functionally diverse than previously thought, and their role in gene expression is under-appreciated. The action of SNORDs in non-methylating complexes can be substituted with oligonucleotides, allowing devising therapies for diseases like Prader-Willi syndrome.
|State||Published - Jun 2017|
Bibliographical noteFunding Information:
We thank Drs. Emanuele Buratti and Dr. Edouard Bertrand for excellent discussions. This work was supported by NIH (R21HD080035 and R21NS098186) and the Foundation for Prader-Willi Research.
© 2017 WILEY Periodicals, Inc.
- Prader-Willi syndrome
- RNA methylation
- RNA processing
- RNA therapy
- alternative splicing
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology (all)