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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RRP1
ribosomal RNA processing 1
Chromosome 21 · 21q22.3
NCBI Gene: 8568Ensembl: ENSG00000160214.14HGNC: HGNC:18785UniProt: P56182
115PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA bindingnucleolusprotein bindingchromosomeneurodegenerative diseasebreast ductal adenocarcinomamenstrual cycle-dependent periodic feverMalignant hyperthermia
✦AI Summary

RRP1 (ribosomal RNA processing 1) is a multifunctional RNA-binding protein with distinct roles in ribosome biogenesis and immune regulation. In ribosome biogenesis, RRP1 localizes to the nucleolus and is essential for early pre-rRNA processing, specifically catalyzing site 2 cleavage in internal transcribed spacer 1 (ITS1) during the conversion of the 90S pre-ribosomal particle into pre-40S and pre-60S subunits 1. This function involves competitive binding with fibrillarin and coordination with exonucleolytic activities to generate mature 28S rRNA 1. Beyond ribosome biogenesis, RRP1 functions as a post-transcriptional suppressor of inflammation by binding the nuclear thymidylate synthetase (TYMS) transcript and reducing TYMS expression in inflammatory macrophages, thereby inhibiting one-carbon metabolism-driven inflammation 2. Myeloid-specific RRP1 deficiency causes severe experimental arthritis with elevated pro-inflammatory cytokines, while RRP1 expression in rheumatoid arthritis patients inversely correlates with TYMS and IL-1β levels 2. Additionally, RRP1 homologs display redox-dependent functions in long-term memory formation through gene regulation mechanisms 3, and possess AP-endonuclease-like catalytic activities for nucleotide damage repair 4. These findings establish RRP1 as a critical regulator integrating ribosome biogenesis with metabolic control of inflammation.

Sources cited
1
RRP1 is involved in site 2 cleavage of ITS1 in pre-rRNAs and facilitates splitting of 90S into pre-40S and pre-60S during early ribosome biogenesis
PMID: 25969445
2
RRP1 suppresses inflammatory responses by binding TYMS transcript and inhibiting one-carbon metabolism; RRP1 deficiency causes severe arthritis; inverse correlation between RRP1 and TYMS/IL-1β in rheumatoid arthritis patients
PMID: 40715096
3
RRP1 and its human homolog APE1 regulate long-term memory formation through redox-dependent transcriptional regulation
PMID: 41289397
4
RRP1 possesses AP-endonuclease-like activities with metal ion-dependent exonuclease and endoribonuclease activities
PMID: 35270011
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.49Moderate
breast ductal adenocarcinomaOpen Targets
0.11Weak
menstrual cycle-dependent periodic feverOpen Targets
0.04Suggestive
Malignant hyperthermiaOpen Targets
0.03Suggestive
HypoventilationOpen Targets
0.02Suggestive
KeloidOpen Targets
0.02Suggestive
crystal arthropathyOpen Targets
0.02Suggestive
rheumatoid arthritisOpen Targets
0.02Suggestive
arthritisOpen Targets
0.02Suggestive
infectionOpen Targets
0.01Suggestive
dry eye syndromeOpen Targets
0.01Suggestive
Lyme diseaseOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
neuroblastomaOpen Targets
0.01Suggestive
lung adenocarcinomaOpen Targets
0.01Suggestive
autoimmune diseaseOpen Targets
0.01Suggestive
endometriosisOpen Targets
0.01Suggestive
age-related macular degenerationOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
Spirochaetales InfectionsOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DDX10Shared pathway100%PIN4Shared pathway100%DDX27Shared pathway100%WDR55Shared pathway100%YBEYShared pathway100%ESF1Shared pathway100%
Tissue Expression6 tissues
Liver
100%
Ovary
87%
Lung
65%
Brain
57%
Bone Marrow
50%
Heart
42%
Gene Interaction Network
Click a node to explore
RRP1DDX10PIN4DDX27WDR55YBEYESF1
PROTEIN STRUCTURE
Preparing viewer…
PDB8FKV · 2.47 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.90LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.68 [0.52–0.90]
RankingsWhere RRP1 stands among ~20K protein-coding genes
  • #4,133of 20,598
    Most Researched115 · top quartile
  • #8,073of 17,882
    Most Constrained (LOEUF)0.90
Genes detectedRRP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The RNA-binding protein RRP1 brakes macrophage one-carbon metabolism to suppress autoinflammation.
PMID: 40715096
Nat Commun · 2025
1.00
2
Rrp1, Rrp2 and Uls1 - Yeast SWI2/SNF2 DNA dependent translocases in genome stability maintenance.
PMID: 35716431
DNA Repair (Amst) · 2022
0.90
3
Gene Regulation and Transcriptomics.
PMID: 33300497
Curr Issues Mol Biol · 2021
0.80
4
PMID: 31932509
J Cell Sci · 2020
0.70
5
Human nucleolar protein Nop52 (RRP1/NNP-1) is involved in site 2 cleavage in internal transcribed spacer 1 of pre-rRNAs at early stages of ribosome biogenesis.
PMID: 25969445
Nucleic Acids Res · 2015
0.60