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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SETD3
SET domain containing 3, actin N3(tau)-histidine methyltransferase
Chromosome 14 · 14q32.2
NCBI Gene: 84193Ensembl: ENSG00000183576.14HGNC: HGNC:20493UniProt: Q86TU7
53PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein-lysine N-methyltransferase activityprotein bindingprotein-L-histidine N-tele-methyltransferase activityactin filament organizationatrial fibrillationbreast cancerHydrocephalusviral disease
✦AI Summary

SETD3 is a protein methyltransferase that primarily functions as an actin-specific histidine N-methyltransferase, catalyzing 3-methylhistidine modification at histidine 73 of actin 12. This histidine methylation contributes to cytoskeleton integrity and is required for smooth muscle contraction during uterine labor 1. The enzyme contains a catalytic SET domain for methyl transfer from S-adenosyl-L-methionine and a RuBisCO LSMT domain for substrate recognition 1. Beyond its methyltransferase activity, SETD3 plays critical roles in viral pathogenesis, particularly for enteroviruses including EV-A71 and EV-D68 3. The viral 2A protease specifically interacts with SETD3's central cleft, disrupting SETD3-actin interactions and facilitating viral RNA replication independent of SETD3's methylation activity 43. Recent evidence suggests SETD3 may also methylate other substrates, including CHD1 at lysine 209, which enhances protein stability and promotes TNF-NFκB pathway activation 5. SETD3 has been implicated in muscle hypertrophy memory through epigenetic mechanisms 6 and various cancer processes 7, indicating functions beyond its established role as an actin histidine methyltransferase 8.

Sources cited
1
SETD3 is an actin-specific histidine methyltransferase with SET and RuBisCO LSMT domains
PMID: 34685411
2
SETD3 catalyzes histidine methylation and contributes to cytoskeleton integrity
PMID: 32188384
3
SETD3 is essential for enterovirus replication through interaction with viral 2A protease
PMID: 31527793
4
Viral 2A protease disrupts SETD3-actin interaction by occupying SETD3's central cleft
PMID: 38755176
5
SETD3 dimethylates CHD1 at lysine 209, enhancing protein stability and TNF-NFκB signaling
PMID: 41045985
6
SETD3 is involved in muscle hypertrophy memory through epigenetic mechanisms
PMID: 29382913
7
SETD3 has potential pathological relevance in human cancers
PMID: 33157163
8
SETD3 functions beyond its histidine methyltransferase activity
PMID: 39299385
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.27Weak
breast cancerOpen Targets
0.07Suggestive
HydrocephalusOpen Targets
0.07Suggestive
viral diseaseOpen Targets
0.06Suggestive
neoplasmOpen Targets
0.06Suggestive
hepatocellular carcinomaOpen Targets
0.05Suggestive
heart diseaseOpen Targets
0.05Suggestive
atrial heart septal defectOpen Targets
0.05Suggestive
exostosisOpen Targets
0.04Suggestive
lung cancerOpen Targets
0.04Suggestive
systemic lupus erythematosusOpen Targets
0.04Suggestive
cervical cancerOpen Targets
0.03Suggestive
spondyloepiphyseal dysplasia tardaOpen Targets
0.03Suggestive
isolated osteopoikilosisOpen Targets
0.03Suggestive
tibial hemimeliaOpen Targets
0.03Suggestive
fibular hemimeliaOpen Targets
0.03Suggestive
metaphyseal dysplasia, Braun-Tinschert typeOpen Targets
0.03Suggestive
Eiken syndromeOpen Targets
0.03Suggestive
infectionOpen Targets
0.03Suggestive
triple-negative breast cancerOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ACTG1Protein interaction92%ACTBProtein interaction91%ACTA1Protein interaction90%ACTA2Protein interaction90%ACTC1Protein interaction90%ACTG2Protein interaction90%
Tissue Expression6 tissues
Heart
100%
Brain
55%
Lung
42%
Liver
32%
Bone Marrow
24%
Ovary
23%
Gene Interaction Network
Click a node to explore
SETD3ACTG1ACTBACTA1ACTA2ACTC1ACTG2
PROTEIN STRUCTURE
Preparing viewer…
PDB6MBK · 1.69 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.48Moderately Constrained
pLIⓘ
0.99Intolerant
Observed/Expected LoF0.32 [0.22–0.48]
RankingsWhere SETD3 stands among ~20K protein-coding genes
  • #8,488of 20,598
    Most Researched53
  • #2,781of 17,882
    Most Constrained (LOEUF)0.48 · top quartile
Genes detectedSETD3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Enterovirus pathogenesis requires the host methyltransferase SETD3.
PMID: 31527793
Nat Microbiol · 2019
1.00
2
The EV71 2A protease occupies the central cleft of SETD3 and disrupts SETD3-actin interaction.
PMID: 38755176
Nat Commun · 2024
0.90
3
SETD3 functions beyond histidine methylation.
PMID: 39299385
Life Sci · 2024
0.80
4
Human Skeletal Muscle Possesses an Epigenetic Memory of Hypertrophy.
PMID: 29382913
Sci Rep · 2018
0.70
5
Protein Histidine Methylation.
PMID: 32188384
Curr Protein Pept Sci · 2020
0.60