The SNORD115 (H/MBII-52) and SNORD116 (H/MBII-85) gene clusters at the imprinted Prader-Willi locus generate canonical box C/D snoRNAs

• Published in: Nucleic acids research Vol. 40; no. 14; pp. 6800 - 6807
• Main Authors: Bortolin-Cavaille, M.-L., Cavaille, J.
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.08.2012
• Subjects: Animals; Base Sequence; Biochemistry, Molecular Biology; Genetic Loci; Genomic Imprinting; Humans; Life Sciences; Mice; Molecular Sequence Data; Multigene Family; Prader-Willi Syndrome - genetics; RNA; RNA, Small Nucleolar - chemistry; RNA, Small Nucleolar - genetics; RNA, Small Nucleolar - metabolism
• ISSN: 0305-1048; 1362-4962
• DOI: 10.1093/nar/gks321

The imprinted Snurf-Snrpn chromosomal domain contains two large arrays of tandemly repeated, paternally expressed box C/D small-nucleolar RNA (snoRNA) genes: the SNORD115 (H/MBII-52) and SNORD116 (H/MBII-85) gene clusters believed to play key roles in the fine-tuning of serotonin receptor (5-HT2C) pre-mRNA processing and in the etiology of the Prader-Willi Syndrome (PWS), respectively. SNORD115 and SNORD116 were recently proposed to undergo significant conversion into shorter RNA species, the so-called psnoRNAs. Here, we provide evidence that argues against the existence of abundant psnoRNAs in human or mouse brain. Instead, we characterize a previously unsuspected low-abundance, fibrillarin-associated SNORD115-derived smaller RNA species. Based on these findings, we strongly recommend that PWS-encoded SNORD115 and SNORD116 be considered as bona fide box C/D snoRNAs.