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9 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RPRD2
regulation of nuclear pre-mRNA domain containing 2
Chromosome 1 · 1q21.2
NCBI Gene: 23248Ensembl: ENSG00000163125.17HGNC: HGNC:29039UniProt: Q5VT52
92PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
transcription preinitiation complexRNA polymerase II complex bindingmRNA 3'-end processingnucleoplasmAbnormality of the skeletal systematopic eczemaprostate carcinomaosteoarthritis, hip
✦AI Summary

RPRD2 (regulation of nuclear pre-mRNA domain containing 2) is a multifunctional protein involved in transcriptional regulation and innate antiviral immunity. As a CTD-interaction domain-containing protein, RPRD2 associates with RNA polymerase II and participates in dephosphorylation of the CTD, contributing to transcription regulation 1. RPRD2 localizes primarily to the nucleus where it binds RNA, DNA, and DNA:RNA hybrids 2. Beyond transcription, RPRD2 functions as a potent innate immune restriction factor (also called REAF—RNA-associated early-stage antiviral factor) that restricts HIV-1 replication by impeding reverse transcription 3. HIV-1 Vpr protein counteracts RPRD2's antiviral activity through proteasomal degradation in a process dependent on Vpr's nuclear localization and interaction with cullin 4A-DCAF1 E3 ligase 3. RPRD2 is upregulated upon T cell activation and highly expressed in myeloid cells 2. Beyond HIV restriction, RPRD2 interacts with HUSH and PAF1 complexes to negatively regulate genome-wide transcription and maintain HIV latency 2. Dysregulation of RPRD2 methylation has been associated with renal aging and functional decline 4, and mutations in RPRD2 were identified in Burkitt lymphoma 5.

Sources cited
1
RPRD2 localizes to nucleus, binds RNA/DNA/DNA:RNA hybrids, restricts HIV-1 by impeding reverse transcription, is targeted by Vpr for degradation, and negatively regulates transcription via HUSH/PAF1 complexes
PMID: 37882563
2
RPRD2 (REAF) potently restricts HIV-1 replication; Vpr induces RPRD2 degradation via nuclear localization and cullin 4A-DCAF1 interaction within 30 minutes of infection
PMID: 31776272
3
RPRD2 is a CTD-interaction domain protein that associates with RNA polymerase II and participates in CTD phosphorylation regulation during transcription
PMID: 22231121
4
RPRD2 hypomethylation is associated with renal aging and accelerated renal function decline
PMID: 38879526
5
RPRD2 mutations were identified in endemic Burkitt lymphoma tumors
PMID: 28465297
6
RPRD2 CID domain structure complexes with RNAPII CTD phosphoisoforms, involved in CTD recognition
PMID: 24997600
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.38Weak
atopic eczemaOpen Targets
0.35Weak
prostate carcinomaOpen Targets
0.18Weak
osteoarthritis, hipOpen Targets
0.18Weak
hyperuricemiaOpen Targets
0.17Weak
total hip arthroplastyOpen Targets
0.13Weak
smoking initiationOpen Targets
0.11Weak
insomniaOpen Targets
0.10Weak
circadian rhythmOpen Targets
0.10Suggestive
hypertensionOpen Targets
0.08Suggestive
response to xenobiotic stimulusOpen Targets
0.08Suggestive
Griscelli diseaseOpen Targets
0.07Suggestive
Griscelli disease type 3Open Targets
0.06Suggestive
Griscelli syndrome type 3Open Targets
0.06Suggestive
PainOpen Targets
0.06Suggestive
basal cell carcinomaOpen Targets
0.05Suggestive
sebaceous gland diseaseOpen Targets
0.05Suggestive
Piebald trait - neurologic defectsOpen Targets
0.05Suggestive
piebald trait-neurologic defects syndromeOpen Targets
0.05Suggestive
uncombable hair syndromeOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CPSF4Shared pathway100%CSTF2TShared pathway100%POLR2BProtein interaction100%POLR2AProtein interaction100%POLR2GProtein interaction100%SUPT5HProtein interaction100%
Tissue Expression6 tissues
Brain
100%
Ovary
63%
Bone Marrow
51%
Heart
51%
Lung
34%
Liver
32%
Gene Interaction Network
Click a node to explore
RPRD2CPSF4CSTF2TPOLR2BPOLR2APOLR2GSUPT5H
PROTEIN STRUCTURE
Preparing viewer…
PDB4FLB · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.14Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.08 [0.04–0.14]
RankingsWhere RPRD2 stands among ~20K protein-coding genes
  • #5,215of 20,598
    Most Researched92
  • #195of 17,882
    Most Constrained (LOEUF)0.14 · top 5%
Genes detectedRPRD2
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
The innate immune factor RPRD2/REAF and its role in the Lv2 restriction of HIV.
PMID: 37882563
mBio · 2023
1.00
2
DNA methylation in peripheral blood is associated with renal aging and renal function decline: a national community study.
PMID: 38879526
Clin Epigenetics · 2024
0.89
3
HIV-1 Accessory Protein Vpr Interacts with REAF/RPRD2 To Mitigate Its Antiviral Activity.
PMID: 31776272
J Virol · 2020
0.78
4
Gene expression changes associated with trajectories of psychopathology in a longitudinal cohort of children and adolescents.
PMID: 32184383
Transl Psychiatry · 2020
0.67
5
RPRD1A and RPRD1B are human RNA polymerase II C-terminal domain scaffolds for Ser5 dephosphorylation.
PMID: 24997600
Nat Struct Mol Biol · 2014
0.56