Small nucleolar RNA SNORD116

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Small nucleolar RNA SNORD116
SNORD116 secondary structure.jpg
Predicted secondary structure and sequence conservation of SNORD116
Identifiers
SymbolSNORD116
Alt. SymbolssnoHBII-85; HBII-85
Rfam RF00108
Other data
RNA type Gene; snRNA; snoRNA; CD-box
Domain(s) Eukaryota
GO GO:0006396 GO:0005730
SO SO:0000593
PDB structures PDBe

In molecular biology, 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 modifying 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, there are 29 tandemnly repeated copies of SNORD116, followed by 48 copies of another C/D box snoRNA, SNORD115, in the Prader–Willi syndrome (PWS) region of chromosome 15. [2] Unlike most other snoRNAs, SNORD116 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]

More evidence comes from the discovery of two individuals that share many traits of PWS sufferers, both have atypical microdeletions on chromosome 15q11–13, the intersection of which contains only the SNORD116 snoRNAs. [5] [6] An early paper had suggested SNORD116 did not have a primary role in PWS. [7]

The targets of SNORD116 are unknown, however a bioinformatic screen located 23 possible targets within protein-coding genes, of these a large fraction were found to be alternatively spliced, suggesting a role of SNORD116 in the regulation of alternative splicing. [8]

Related Research Articles

Prader–Willi syndrome Rare genetic disorder caused by part of the fathers chromosome 15 being missing

Prader–Willi syndrome (PWS) is a genetic disorder caused by a loss of function of specific genes on chromosome 15. In newborns, symptoms include weak muscles, poor feeding, and slow development. Beginning in childhood, those affected become constantly hungry, which often leads to obesity and type 2 diabetes. Mild to moderate intellectual impairment and behavioral problems are also typical of the disorder. Often, affected individuals have a narrow forehead, small hands and feet, short height, light skin and hair. Most are unable to have children.

In molecular biology, Small nucleolar RNAs (snoRNAs) are a class of small RNA molecules that primarily guide chemical modifications of other RNAs, mainly ribosomal RNAs, transfer RNAs and small nuclear RNAs. There are two main classes of snoRNA, the C/D box snoRNAs, which are associated with methylation, and the H/ACA box snoRNAs, which are associated with pseudouridylation. SnoRNAs are commonly referred to as guide RNAs but should not be confused with the guide RNAs that direct RNA editing in trypanosomes.

Small nucleolar RNA SNORD101

In molecular biology, snoRNA U101 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD115

In molecular biology, SNORD115 is a non-coding RNA (ncRNA) molecule known as a small nucleolar RNA which usually functions in guiding the modification of other non-coding RNAs. This type of modifying RNA is usually located in the nucleolus of the eukaryotic cell which is a major site of snRNA biogenesis. HBII-52 refers to the human gene, whereas RBII-52 is used for the rat gene and MBII-52 is used for naming the mouse gene.

Small nucleolar RNA SNORD15

In molecular biology, SNORD15 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD19

In molecular biology, SNORD19 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD42

In molecular biology, snoRNA U42 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD53

In molecular biology, snoRNA U53 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD56

In molecular biology, snoRNA U56 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD58

In molecular biology, snoRNA U58 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD64

In molecular biology, SNORD64 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD66

In molecular biology, SNORD66 is a non-coding RNA (ncRNA) molecule which functions in the modification of other small nuclear RNAs (snRNAs). This type of modifying 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.

Small nucleolar RNA SNORD67

In molecular biology, SNORD67 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD100

In molecular biology, Small Nucleolar RNA SNORD100 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD93

In molecular biology, Small Nucleolar RNA SNORD93 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD23

In molecular biology, Small Nucleolar RNA SNORD23 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD75

In molecular biology, Small Nucleolar RNA SNORD75 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD88

In molecular biology, Small Nucleolar RNA SNORD88 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD92

In molecular biology, Small Nucleolar RNA SNORD92 is a non-coding RNA (ncRNA) molecule which functions in the biogenesis (modification) of other small nuclear RNAs (snRNAs). This type of modifying RNA is 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.

Small nucleolar RNA SNORD113

In molecular biology, Small nucleolar RNA SNORD113 is a small nucleolar RNA molecule which is located in the imprinted human 14q32 locus and may play a role in the evolution and/or mechanism of the epigenetic imprinting process.

References

  1. Galardi, S.; Fatica, A.; Bachi, A.; Scaloni, A.; Presutti, C.; Bozzoni, I. (October 2002). "Purified Box C/D snoRNPs Are Able to Reproduce Site-Specific 2'-O-Methylation of Target RNA in Vitro". Molecular and Cellular Biology . 22 (19): 6663–6668. doi:10.1128/MCB.22.19.6663-6668.2002. PMC   134041 . PMID   12215523.
  2. 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. Bibcode:2000PNAS...9714311C. doi: 10.1073/pnas.250426397 . PMC   18915 . PMID   11106375.
  3. 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. PMC   2323313 . PMID   18166085.
  4. Ding F, Li HH, Zhang S, et al. (2008). Akbarian S (ed.). "SnoRNA Snord116 (Pwcr1/MBII-85) Deletion Causes Growth Deficiency and Hyperphagia in Mice". PLOS ONE. 3 (3): e1709. Bibcode:2008PLoSO...3.1709D. doi: 10.1371/journal.pone.0001709 . PMC   2248623 . PMID   18320030.
  5. Sahoo T, del Gaudio D, German JR, Shinawi M, Peters SU, Person RE, Garnica A, Cheung SW, Beaudet AL (2008). "Prader-Willi phenotype caused by paternal deficiency for the HBII-85 C/D box small nucleolar RNA cluster". Nat Genet. 40 (6): 719–21. doi:10.1038/ng.158. PMC   2705197 . PMID   18500341.
  6. de Smith AJ, Purmann C, Walters RG, et al. (June 2009). "A deletion of the HBII-85 class of small nucleolar RNAs (snoRNAs) is associated with hyperphagia, obesity and hypogonadism". Hum. Mol. Genet. 18 (17): 3257–65. doi:10.1093/hmg/ddp263. PMC   2722987 . PMID   19498035.
  7. Runte M, Varon R, Horn D, Horsthemke B, Buiting K (2005). "Exclusion of the C/D box snoRNA gene cluster HBII-52 from a major role in Prader-Willi syndrome". Hum Genet. 116 (3): 228–30. doi:10.1007/s00439-004-1219-2. PMID   15565282. S2CID   23190709.
  8. Bazeley PS, Shepelev V, Talebizadeh Z, Butler MG, Fedorova L, Filatov V, Fedorov A (2008). "snoTARGET shows that human orphan snoRNA targets locate close to alternative splice junctions". Gene. 408 (1–2): 172–9. doi:10.1016/j.gene.2007.10.037. PMC   6800007 . PMID   18160232.