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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HEMK2
HemK methyltransferase 2, ETF1 glutamine and histone H4 lysine
Chromosome 21 · 21q21.3
NCBI Gene: 29104Ensembl: ENSG00000156239.13HGNC: HGNC:16021UniProt: Q9Y5N5
33PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
site-specific DNA-methyltransferase (adenine-specific) activitypositive regulation of cell growtharsonoacetate metabolic processmethylationKeloidgestational diabetesadolescent idiopathic scoliosispoisoning
✦AI Summary

HEMK2 is a multifunctional methyltransferase that catalyzes post-translational modifications on diverse substrates. As the catalytic subunit of a heterodimer with TRMT112, HEMK2 monomethylates histone H4 lysine 12 (H4K12me1), a modification enriched at promoters of cell cycle regulator genes 1. HEMK2 also catalyzes N5-methylation of glutamine residues within the conserved GGQ motif of eukaryotic release factor 1 (eRF1) and other proteins containing a Gly-Gln-X3-Arg sequence context 23. ETF1 methylation requires its complexation with ERF3 in the GTP-bound state 4. Recent structural studies reveal HEMK2 utilizes distinct substrate-binding pockets to accommodate both lysine and glutamine residues, though it exhibits stronger preference for glutamine methylation 5. Beyond protein modification, HEMK2 participates in arsenic metabolism by converting monomethylarsonous acid to less toxic dimethylarsonic acid, though this represents a limited role compared to AS3MT 6. Clinically, HEMK2 depletion impairs proliferation of androgen-dependent and castration-resistant prostate cancer cells, linking H4K12 methylation to cancer cell growth regulation 1. TRMT112 regulates HEMK2 expression through quality control mechanisms, ensuring only functional isoforms persist in cells 7.

Sources cited
1
HEMK2-TRMT112 heterodimer catalyzes H4K12me1 at cell cycle regulator promoters and regulates prostate cancer cell proliferation
PMID: 31061526
2
HEMK2 requires GQX3R motif for glutamine methylation and methylates 58 novel peptide substrates including CHD5 and NUT
PMID: 26797129
3
Structural characterization reveals HEMK2-TRMT112 complex recognizes glutamine substrates through specific binding pocket
PMID: 32969463
4
HEMK2 exhibits distinct sequence specificities for Q-methylation (G-Q-X3-R) versus K-methylation (S/T-K context) with preference for glutamine
PMID: 38284488
5
HEMK2 methylates glutamine, lysine, deoxyadenosine, and arsenicals; conserved across eukaryotes, archaea, and bacteria
PMID: 35621100
6
TRMT112 regulates HEMK2 isoform expression through quality control, eliminating nonfunctional isoforms
PMID: 31466382
7
HEMK2 methylates ETF1 on Gln-185; ETF1 must be complexed to ERF3 in GTP-bound form for efficient methylation
PMID: 18539146
8
HEMK2-TRMT112 is inhibited by NTMT1 bisubstrate inhibitors, representing first potent inhibitor for HEMK2/KMT9
PMID: 34192867
Disease Associationsⓘ20
KeloidOpen Targets
0.28Weak
gestational diabetesOpen Targets
0.22Weak
adolescent idiopathic scoliosisOpen Targets
0.20Weak
poisoningOpen Targets
0.20Weak
glaucomaOpen Targets
0.20Weak
facial morphologyOpen Targets
0.19Weak
placenta praeviaOpen Targets
0.12Weak
cancerOpen Targets
0.09Suggestive
breast cancerOpen Targets
0.08Suggestive
DermatochalasisOpen Targets
0.08Suggestive
glomerulonephritisOpen Targets
0.08Suggestive
hyperpituitarismOpen Targets
0.07Suggestive
posterior cortical atrophyOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
lung cancerOpen Targets
0.06Suggestive
gastrointestinal stromal tumorOpen Targets
0.04Suggestive
prostate cancerOpen Targets
0.03Suggestive
Cognitive impairmentOpen Targets
0.02Suggestive
non-small cell lung carcinomaOpen Targets
0.02Suggestive
lung adenocarcinomaOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
H4C9Protein interaction98%H4C2Protein interaction98%H4C4Protein interaction98%H4C5Protein interaction98%H4C13Protein interaction98%H4C11Protein interaction98%
Tissue Expression6 tissues
Ovary
100%
Heart
84%
Liver
38%
Brain
30%
Lung
28%
Bone Marrow
17%
Gene Interaction Network
Click a node to explore
HEMK2H4C9H4C2H4C4H4C5H4C13H4C11
PROTEIN STRUCTURE
Preparing viewer…
PDB8QDG · 1.39 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.92LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.44 [0.92–1.92]
RankingsWhere HEMK2 stands among ~20K protein-coding genes
  • #11,348of 20,598
    Most Researched33
  • #17,361of 17,882
    Most Constrained (LOEUF)1.92
Genes detectedHEMK2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
[HEMK-Like Methyltransferases in the Regulation of Cellular Processes].
PMID: 35621100
Mol Biol (Mosk) · 2022
1.00
2
Mammalian HEMK1 methylates glutamine residue of the GGQ motif of mitochondrial release factors.
PMID: 35260756
Sci Rep · 2022
0.90
3
Structural insight into HEMK2-TRMT112-mediated glutamine methylation.
PMID: 32969463
Biochem J · 2020
0.80
4
Substrate Specificity of the HEMK2 Protein Glutamine Methyltransferase and Identification of Novel Substrates.
PMID: 26797129
J Biol Chem · 2016
0.70
5
Distinct specificities of the HEMK2 protein methyltransferase in methylation of glutamine and lysine residues.
PMID: 38284488
Protein Sci · 2024
0.60