SNORD3A

Organism: Homo sapiens
Review Status: COMPLETE
Aliases:
U3 snoRNA U3 Small nucleolar RNA U3
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Gene Description

SNORD3A (U3 snoRNA) is a C/D box small nucleolar RNA essential for ribosome biogenesis. Unlike typical C/D box snoRNAs that guide 2'-O-methylation, U3 snoRNA guides site-specific cleavage of pre-rRNA through base-pairing interactions. It is a critical component of the SSU-processome complex that directs maturation of 18S rRNA and assembly of the 40S small ribosomal subunit. This gene is also known as U3 snoRNA and is atypical among C/D box snoRNAs because it guides pre-rRNA cleavage rather than 2'-O-methylation.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0042134 rRNA primary transcript binding
TAS
PMID:21349877
The initial U3 snoRNA:pre-rRNA base pairing interaction requ...
ACCEPT
Summary: U3 snoRNA binds to pre-rRNA through multiple base-pairing interactions that are essential for pre-18S rRNA folding
Reason: This is a core molecular function of U3 snoRNA well-supported by experimental evidence showing direct base-pairing with rRNA primary transcripts
Supporting Evidence:
PMID:21349877
the U3 snoRNA is required for folding of the pre-18S rRNA by in vivo chemical probing
GO:0070181 small ribosomal subunit rRNA binding
TAS
PMID:31996908
Synergistic defects in pre-rRNA processing from mutations in...
ACCEPT
Summary: U3 snoRNA binds specifically to small ribosomal subunit (18S) rRNA sequences during ribosome biogenesis
Reason: Well-established function of U3 in binding SSU rRNA, distinct from its role in binding the primary transcript
Supporting Evidence:
PMID:31996908
U3 snoRNA and the associated Rrp9/U3-55K protein are essential for 18S rRNA production by the SSU-processome complex
GO:0030490 maturation of SSU-rRNA
TAS
PMID:31996908
Synergistic defects in pre-rRNA processing from mutations in...
ACCEPT
Summary: U3 snoRNA is essential for maturation of the small ribosomal subunit rRNA through directing pre-rRNA cleavages at sites A0, A1, and A2
Reason: Core biological process function with extensive experimental support showing U3 is required for SSU-rRNA maturation
Supporting Evidence:
PMID:31996908
U3 and Rrp9 are required for early pre-rRNA cleavages at sites A0, A1 and A2
GO:0006364 rRNA processing
TAS
PMID:21349877
The initial U3 snoRNA:pre-rRNA base pairing interaction requ...
ACCEPT
Summary: U3 snoRNA guides site-specific cleavage of pre-rRNA, a critical step in rRNA processing
Reason: Fundamental role of U3 in rRNA processing through cleavage guidance rather than the typical methylation guidance of other C/D box snoRNAs
Supporting Evidence:
file:human/SNORD3A/SNORD3A-deep-research-manual.md
Unlike typical C/D box snoRNAs that guide 2'-O-methylation, U3 snoRNA plays an essential and unique role in guiding site-specific cleavage of pre-ribosomal RNA during ribosome biogenesis
PMID:21349877
Feb 23. The initial U3 snoRNA:pre-rRNA base pairing interaction required for pre-18S rRNA folding revealed by in vivo chemical probing.

Core Functions

Essential guide RNA for maturation of 18S rRNA via base-pairing with pre-rRNA

Directly Involved In:
Supporting Evidence:
  • PMID:21349877
    We tested whether the U3 snoRNA is required for folding of the pre-18S rRNA by in vivo chemical probing with DMS when the U3 snoRNA is conditionally expressed, and determined that there were indeed changes in the structure of the pre-rRNA dependent on the presence of the U3 snoRNA (Figure 1B)
  • PMID:31996908
    U3 and Rrp9 are required for early pre-rRNA cleavages at sites A0, A1 and A2, but the mechanism remains unclear

Guides site-specific cleavage of pre-rRNA (not methylation, unlike other C/D box snoRNAs)

Directly Involved In:
Supporting Evidence:
  • PMID:31996908
    In contrast, the U3 box C/D snoRNP lacks a known role in RNA methylation but is essential for pre-rRNA cleavage at sites A0, A1 and A2.

References

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Suggested Questions for Experts

Q: What are the complete set of protein partners that interact with U3 snoRNA during different stages of ribosome biogenesis?

Q: Are there tissue-specific or developmental stage-specific variants of U3 snoRNA expression or modification that affect ribosome heterogeneity?

Q: How does the observed correlation between SNORD3A levels and prion disease progression relate to ribosome dysfunction or cellular stress pathways?

Suggested Experiments

Experiment: Use SHAPE-MaP or other RNA structure probing methods to map the complete secondary and tertiary structure of human U3 snoRNA in complex with processome proteins

Hypothesis: U3 snoRNA structural domains beyond known base-pairing regions contribute to SSU-processome assembly

Type: Structural analysis

Experiment: Generate human cell lines with specific U3 snoRNA mutations targeting different functional domains and assess pre-rRNA processing kinetics, ribosome production rates, and stress response pathways

Hypothesis: U3 snoRNA variants affect ribosome biogenesis efficiency and cellular stress responses

Type: Functional assay

Deep Research

Manual

(SNORD3A-deep-research-manual.md)

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πŸ“š Additional Documentation

Notes

(SNORD3A-notes.md)

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Readme

(README.md)

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πŸ“„ View Raw YAML

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