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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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NOP53
NOP53 ribosome biogenesis factor
Chromosome 19 · 19q13.33
NCBI Gene: 29997Ensembl: ENSG00000105373.20HGNC: HGNC:4333UniProt: Q9NZM5
136PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA RepairHub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
rDNA heterochromatinp53 bindingRNA bindingprotein bindingneoplasmgastric canceremphysemapapillary thyroid carcinoma
✦AI Summary

NOP53 is a multifunctional nucleolar protein essential for ribosome biogenesis and cellular stress response. Primarily, NOP53 integrates the 5S ribosomal ribonucleoprotein (RNP) into the large ribosomal subunit and participates in 5.8S rRNA maturation through interaction with the MTR4 helicase-exosome complex via its arch-interacting motif 12. Beyond ribosome assembly, NOP53 functions as a nucleolar stress sensor: upon DNA damage or ribosome biogenesis perturbation, disrupted NOP53-RPL11 interaction permits RPL11 nuclear translocation, which inhibits MDM2 and activates p53/TP53 1. NOP53 also stabilizes p53 through direct interaction, preventing MDM2-mediated degradation 3. Additional tumor-suppressive functions include positive regulation of PTEN stability, antagonizing PI3K-AKT signaling, and suppression of autophagy through ZKSCAN3-dependent and -independent pathways 45. Clinically, NOP53 dysregulation associates with cancer development: downregulation causes chr19 instability and abnormal nuclear morphology in cervical cancer cells 6, while germline variants may increase familial non-medullary thyroid cancer susceptibility 7. Conversely, viruses exploit cytoplasmic NOP53 translocation to suppress interferon-beta production and enhance replication 89.

Sources cited
1
NOP53 integrates 5S RNP into large ribosomal subunit and acts as nucleolar sensor regulating p53 activation
PMID: 24120868
2
PICT1/NOP53 interacts with MTR4 exosome complex via AIM motif to regulate pre-rRNA processing steps
PMID: 36403484
3
NOP53 stabilizes p53 through direct interaction, preventing MDM2-dependent degradation
PMID: 22522597
4
NOP53 positively regulates PTEN stability, antagonizing PI3K-AKT signaling pathway
PMID: 15355975
5
NOP53 suppresses autophagy through ZKSCAN3-dependent transcriptional activation and histone H3 dephosphorylation
PMID: 34502226
6
NOP53 downregulation causes chromosomal instability, abnormal nuclear morphology, and mitotic defects in cervical cancer
PMID: 30421090
7
Germline NOP53 variant associates with familial non-medullary thyroid cancer susceptibility as low-penetrant modifier
PMID: 31703244
8
Cytoplasmic NOP53 translocation during viral infection suppresses RIG-I and attenuates interferon-beta production
PMID: 29677136
9
Viral protein γ34.5 manipulates NOP53 translocation to facilitate eIF2α dephosphorylation for HSV-1 replication
PMID: 29367603
Disease Associationsⓘ20
neoplasmOpen Targets
0.10Suggestive
gastric cancerOpen Targets
0.08Suggestive
emphysemaOpen Targets
0.07Suggestive
papillary thyroid carcinomaOpen Targets
0.07Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
viral diseaseOpen Targets
0.05Suggestive
cervical cancerOpen Targets
0.05Suggestive
atrial fibrillationOpen Targets
0.04Suggestive
infectionOpen Targets
0.04Suggestive
breast cancerOpen Targets
0.03Suggestive
hepatocellular carcinomaOpen Targets
0.03Suggestive
prostate cancerOpen Targets
0.03Suggestive
gliomaOpen Targets
0.03Suggestive
endometrial cancerOpen Targets
0.03Suggestive
cancerOpen Targets
0.02Suggestive
oral squamous cell carcinomaOpen Targets
0.02Suggestive
glioblastomaOpen Targets
0.02Suggestive
mycosis fungoidesOpen Targets
0.02Suggestive
obesityOpen Targets
0.02Suggestive
major depressive disorderOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RPL12Protein interaction100%RPL38Protein interaction100%RPL29Protein interaction100%RPL32Protein interaction100%RPL31Protein interaction100%RPL3Protein interaction100%
Tissue Expression6 tissues
Ovary
100%
Lung
35%
Bone Marrow
31%
Liver
25%
Brain
15%
Heart
11%
Gene Interaction Network
Click a node to explore
NOP53RPL12RPL38RPL29RPL32RPL31RPL3
PROTEIN STRUCTURE
Preparing viewer…
PDB8FLE · 2.48 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.08LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.81 [0.62–1.08]
RankingsWhere NOP53 stands among ~20K protein-coding genes
  • #3,414of 20,598
    Most Researched136 · top quartile
  • #10,926of 17,882
    Most Constrained (LOEUF)1.08
Genes detectedNOP53
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Downregulation of NOP53 Ribosome Biogenesis Factor Leads to Abnormal Nuclear Division and Chromosomal Instability in Human Cervical Cancer Cells.
PMID: 30421090
Pathol Oncol Res · 2020
1.00
2
SMALL ORGAN4 Is a Ribosome Biogenesis Factor Involved in 5.8S Ribosomal RNA Maturation.
PMID: 32913045
Plant Physiol · 2020
0.90
3
MTR4 adaptor PICT1 functions in two distinct steps during pre-rRNA processing.
PMID: 36403484
Biochem Biophys Res Commun · 2022
0.80
4
PMID: 31703244
Genes (Basel) · 2019
0.70
5
Cytoplasmic Translocation of Nucleolar Protein NOP53 Promotes Viral Replication by Suppressing Host Defense.
PMID: 29677136
Viruses · 2018
0.60