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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZC3H3
zinc finger CCCH-type containing 3
Chromosome 8 · 8q24.3
NCBI Gene: 23144Ensembl: ENSG00000014164.8HGNC: HGNC:28972UniProt: Q8IXZ2
30PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleusnucleoplasmRNA catabolic processmRNA cleavage and polyadenylation specificity factor complexneurodegenerative diseasejaw diseaseCerebral degenerationglomerulonephritis
✦AI Summary

ZC3H3 (zinc finger CCCH-type containing 3) is a nuclear RNA-binding protein that plays critical roles in mRNA export and RNA decay pathways. The protein is required for the export of polyadenylated mRNAs from the nucleus, functioning as a coupling factor between mRNA processing and nuclear export 1. ZC3H3 serves as an essential component of the Poly(A) Tail eXosome Targeting (PAXT) complex, working alongside MTR4, ZFC3H1, RBM26, and RBM27 to facilitate nuclear RNA decay 2. Recent studies reveal ZC3H3's involvement in a sophisticated RNA sorting mechanism where it helps trigger ZFC3H1 conformational changes that activate exosomal degradation of short RNAs with fewer exons, while allowing longer RNAs to proceed to nuclear export 3. Additionally, ZC3H3 functions as an m6A methylation writer, contributing to RNA modification processes 4. The protein demonstrates functional conservation across species, as depletion in both Drosophila and human cells results in mRNA export defects and altered nuclear RNA distribution 1. ZC3H3 has potential clinical significance, with altered expression and methylation patterns observed in various cancers including adrenocortical carcinoma and hepatocellular carcinoma 56.

Sources cited
1
ZC3H3 is required for export of polyadenylated mRNAs and couples mRNA processing with export
PMID: 19364924
2
ZC3H3 is an essential component of the PAXT complex for nuclear RNA decay
PMID: 31950173
3
ZC3H3 helps trigger ZFC3H1 conformational changes for selective RNA degradation
PMID: 39461342
4
ZC3H3 functions as an m6A methylation writer
PMID: 33611339
5
ZC3H3 expression patterns are altered in adrenocortical carcinoma
PMID: 33725886
6
ZC3H3 methylation and transcription changes are associated with hepatocellular carcinoma
PMID: 38302914
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.52Moderate
jaw diseaseOpen Targets
0.07Suggestive
Cerebral degenerationOpen Targets
0.05Suggestive
glomerulonephritisOpen Targets
0.03Suggestive
hepatocellular carcinomaOpen Targets
0.03Suggestive
female reproductive system diseaseOpen Targets
0.02Suggestive
breast cancerOpen Targets
0.01Suggestive
adrenal cortex carcinomaOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
non-alcoholic steatohepatitisOpen Targets
0.00Suggestive
acneOpen Targets
0.00Suggestive
CirrhosisOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
liver cancerOpen Targets
0.00Suggestive
liver diseaseOpen Targets
0.00Suggestive
obesityOpen Targets
0.00Suggestive
urinary bladder carcinomaOpen Targets
0.00Suggestive
hepatitis B virus induced hepatocellular carcinomaOpen Targets
0.00Suggestive
Uterine CarcinosarcomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PPP1R8Shared pathway100%STK31Shared pathway100%RBM27Shared pathway100%CPSF2Protein interaction100%CPSF3Protein interaction98%CPSF1Protein interaction97%
Tissue Expression6 tissues
Liver
100%
Lung
57%
Bone Marrow
41%
Ovary
40%
Brain
22%
Heart
15%
Gene Interaction Network
Click a node to explore
ZC3H3PPP1R8STK31RBM27CPSF2CPSF3CPSF1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8IXZ2
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.66LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.50 [0.38–0.66]
RankingsWhere ZC3H3 stands among ~20K protein-coding genes
  • #12,072of 20,598
    Most Researched30
  • #4,816of 17,882
    Most Constrained (LOEUF)0.66
Genes detectedZC3H3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The role of m6A modification in the biological functions and diseases.
PMID: 33611339
Signal Transduct Target Ther · 2021
1.00
2
The human ZC3H3 and RBM26/27 proteins are critical for PAXT-mediated nuclear RNA decay.
PMID: 31950173
Nucleic Acids Res · 2020
0.90
3
Dual modes of ZFC3H1 confer selectivity in nuclear RNA sorting.
PMID: 39461342
Mol Cell · 2024
0.80
4
A conserved CCCH-type zinc finger protein regulates mRNA nuclear adenylation and export.
PMID: 19364924
J Cell Biol · 2009
0.70
5
Expression patterns and prognostic value of m6A RNA methylation regulators in adrenocortical carcinoma.
PMID: 33725886
Medicine (Baltimore) · 2021
0.60