Small nucleolar RNA SNORD116

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Small nucleolar RNA SNORD116

Type:	Gene; snRNA; guide; CD-box;
2° structure:	Predicted; PFOLD; Daub J
Seed alignment:	Griffiths-Jones SR
Avg length:	92.2 nucleotides
Avg identity:	80%

SNORD116 (also known as HBII-85) is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifiying RNA is usually located in the nucleolus of the eukaryotic cell which is a major site of snRNA biogenesis. It is known as a small nucleolar RNA (snoRNA) and also often referred to as a guide RNA.

SNORD116 belongs to the C/D box class of snoRNAs which contain the conserved sequence motifs known as the C box (UGAUGA) and the D box (CUGA). Most of the members of the box C/D family function in directing site-specific 2'-O-methylation of substrate RNAs^[1].

In the human genome, HBII-85 is encoded in a tandemly repeated array with another C/D box snoRNA, HBII-52, in the Prader-Willi syndrome (PWS) region of chromosome 15 ^[2]. Unlike most other snoRNAs, HBII-85 is expressed prevalently in the brain (but is absent in PWS patients) and lacks any significant complementarity with ribosomal RNA. Mouse models of PWS show similar symptoms to humans (hyperphagia and growth deficiency), providing further evidence that PWS is directly linked to the deletion of SNORD116^[3]^[4].

[edit] References

^ Galardi, S; Fatica A, Bachi A, Scaloni A, Presutti C, Bozzoni I (2002). "Purified box C/D snoRNPs are able to reproduce site-specific 2'-O-methylation of target RNA in vitro". Mol Cell Biol 22: 6663–6668. doi:10.1128/MCB.22.19.6663-6668.2002. PMID 12215523.
^ Cavaillé J, Buiting K, Kiefmann M, et al (2000). "Identification of brain-specific and imprinted small nucleolar RNA genes exhibiting an unusual genomic organization". Proc. Natl. Acad. Sci. U.S.A. 97 (26): 14311–6. doi:10.1073/pnas.250426397. PMID 11106375.
^ Skryabin BV, Gubar LV, Seeger B, et al (2007). "Deletion of the MBII-85 snoRNA gene cluster in mice results in postnatal growth retardation". PLoS Genet. 3 (12): e235. doi:10.1371/journal.pgen.0030235. PMID 18166085.
^ Ding F, Li HH, Zhang S, et al (2008). "SnoRNA Snord116 (Pwcr1/MBII-85) Deletion Causes Growth Deficiency and Hyperphagia in Mice". PLoS ONE 3 (3): e1709. doi:10.1371/journal.pone.0001709. PMID 18320030.