Affinage

HEMK2

Methyltransferase HEMK2 · UniProt Q9Y5N5

Length
214 aa
Mass
23.0 kDa
Annotated
2026-06-10
20 papers in source corpus 13 papers cited in narrative 13 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

HEMK2 is an AdoMet-dependent protein methyltransferase whose deeply conserved and best-supported function is the N5-monomethylation of the glutamine in the GGQ motif of class I translation release factors, a modification required for efficient peptide chain release and proper translation termination (PMID:18539146, PMID:38881530). This activity is structurally rooted in a canonical Rossmann-fold methyltransferase domain in which the conserved NPPY motif orients the substrate glutamine nitrogen toward the AdoMet methyl group, a catalytic logic first established for the bacterial ortholog PrmC/HemK and conserved through human HEMK2 (PMID:12741815, PMID:16364916, PMID:32969463). In eukaryotes HEMK2 functions as an obligate heterodimer with TRMT112 and methylates Gln185 of eRF1, with the human catalytic subunit able to complement loss of the yeast ortholog (PMID:18539146, PMID:31061526). The functional consequence of this modification is clearest in vivo: loss of eRF1 glutamine methylation causes ribosomal stalling, disome formation, No-Go Decay activation, mRNA degradation and apoptosis, establishing eRF1 as the primary functional substrate (PMID:38881530). Beyond eRF1, HEMK2 has broad in vitro substrate scope and methylates additional protein substrates (PMID:26797129), and the heterodimer (as KMT9) has been linked to histone H4K12 monomethylation, promoter occupancy at cell-cycle genes, and prostate cancer proliferation (PMID:31061526), although biochemical profiling shows HEMK2 strongly prefers glutamine over lysine substrates and attributes the bulk of cellular H4K12me1 to SETD6 rather than HEMK2 (PMID:38284488). In prostate cancer cells HEMK2/KMT9 additionally localizes to mitochondria and monomethylates DLAT at Lys596 to regulate pyruvate dehydrogenase complex activity, de novo lipogenesis and proliferation (PMID:39885202). Structure-based bisubstrate inhibitors targeting both the SAM and substrate pockets impair KMT9 target-gene expression and proliferation of therapy-resistant prostate cancer cells (PMID:38167811).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2003 High

    Established the catalytic mechanism of GGQ-motif glutamine methylation, defining how an AdoMet methyltransferase positions a glutamine substrate for N5 methyl transfer.

    Evidence X-ray crystallography of bacterial PrmC/HemK apo, substrate and product complexes with active-site analysis

    PMID:12741815

    Open questions at the time
    • Bacterial enzyme only; eukaryotic HEMK2 heterodimer not yet addressed
    • No partner protein (TRMT112) in the structure
  2. 2005 High

    Identified release factor RF1 as the physiological substrate and showed methylation stimulates peptide release, linking the modification to translation termination.

    Evidence Crystal structure of RF1–PrmC complex plus methylation and mutagenesis assays; complementation in C. trachomatis ortholog

    PMID:15629922 PMID:16364916

    Open questions at the time
    • Demonstrated in bacteria; eukaryotic eRF1 substrate not yet confirmed
    • Quantitative effect on termination kinetics in vivo not measured
  3. 2006 Medium

    Tested and ruled out a proposed DNA N6-adenine methyltransferase activity for mammalian HEMK2, redirecting attention to protein substrates.

    Evidence Subcellular localization, in vitro methyltransferase assay, and sensitive mass spectrometry detection of genomic m6A in mouse

    PMID:16684535

    Open questions at the time
    • Negative result does not identify the true substrate
    • Single lab
  4. 2008 High

    Established the conserved eukaryotic function: human HEMK2 with TRMT112 methylates eRF1 and complements the yeast ortholog, extending the bacterial termination-factor role to mammals.

    Evidence In vitro methylation with recombinant HEMK2–TRMT112 and yeast mtq2Δ complementation

    PMID:18539146

    Open questions at the time
    • Cellular consequences of eRF1 methylation in mammals not yet defined
    • Whether TRMT112 is obligate not yet resolved
  5. 2016 High

    Defined HEMK2 sequence specificity (GQX3R) and revealed a broad in vitro substrate repertoire beyond eRF1, including cellular methylation of CHD5 and NUT.

    Evidence Peptide SPOT array profiling, in vitro protein-domain methylation, and in-cell assays with transfected substrates

    PMID:26797129

    Open questions at the time
    • Physiological relevance of non-eRF1 substrates unconfirmed
    • Stoichiometry and abundance of these modifications in vivo unknown
  6. 2019 High

    Proposed a chromatin role: as KMT9, the obligate HEMK2–TRMT112 heterodimer monomethylates H4K12, occupies cell-cycle gene promoters, and drives prostate cancer proliferation.

    Evidence In vitro HMT assay, KMT9–SAH–H4K12me1 co-crystal structure, ChIP-seq, siRNA knockdown, and xenografts

    PMID:31061526

    Open questions at the time
    • Relative contribution of HEMK2 versus other enzymes to bulk H4K12me1 not addressed
    • Direct link between H4K12me1 and promoter occupancy not mechanistically resolved
  7. 2020 High

    Resolved the structural basis for dual glutamine/lysine recognition, showing a HEMK2 pocket that accommodates and methylates the substrate glutamine.

    Evidence Two crystal structures of HEMK2–TRMT112 with SAM and with SAH/methylglutamine plus mass spectrometry verification

    PMID:32969463

    Open questions at the time
    • Does not establish which activity dominates in cells
    • Single lab
  8. 2024 Medium

    Quantitatively ranked HEMK2 substrate preference, showing strong selectivity for eRF1 glutamine over H4K12 lysine and reassigning most cellular H4K12me1 to SETD6.

    Evidence Peptide SPOT arrays, in vitro protein methylation, and siRNA plus mass spectrometry quantification of H4K12me1 in DU145 cells

    PMID:38284488

    Open questions at the time
    • Does not exclude context-specific HEMK2 H4K12 activity
    • Single cell line and single lab
  9. 2024 High

    Established eRF1 as the primary functional substrate in vivo by linking loss of methylation to ribosome stalling, No-Go Decay and apoptosis.

    Evidence Drosophila germline RNAi, polysome/disome profiling, No-Go Decay rescue, and epistasis with methylation-deficient eRF1

    PMID:38881530

    Open questions at the time
    • Performed in Drosophila germline; mammalian tissue specificity not addressed
    • Whether non-eRF1 substrates contribute to phenotype not tested
  10. 2024 High

    Provided a structure-guided chemical tool: a bisubstrate inhibitor occupying SAM and substrate pockets that suppresses KMT9 target genes and therapy-resistant prostate cancer growth.

    Evidence Structure-based design with co-crystals, biochemical selectivity profiling, target engagement, and proliferation assays; earlier NTMT1 bisubstrate probe pulldown

    PMID:34192867 PMID:38167811

    Open questions at the time
    • On-target gene effects not fully separated from possible off-target activity
    • In vivo efficacy and pharmacology in patients not established
  11. 2025 High

    Uncovered a mitochondrial role specific to prostate cancer: HEMK2/KMT9 methylates DLAT K596 to regulate pyruvate dehydrogenase complex activity, lipogenesis and proliferation.

    Evidence Subcellular fractionation, in vitro/in vivo methylation mapping DLAT K596, PDC activity and lipogenesis assays, siRNA, xenografts, and patient tissue correlation

    PMID:39885202

    Open questions at the time
    • Mechanism of mitochondrial targeting of the heterodimer unknown
    • Generality beyond prostate cancer not established

Open questions

Synthesis pass · forward-looking unresolved questions
  • It remains unresolved how HEMK2 partitions among its glutamine, lysine and mitochondrial substrates across normal tissues and which activities dominate in physiological versus oncogenic settings.
  • No quantitative substrate flux measured in normal mammalian tissues
  • Determinants of nuclear versus mitochondrial localization unknown
  • Relative physiological weight of eRF1, H4K12 and DLAT methylation undefined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016740 transferase activity 4 GO:0140096 catalytic activity, acting on a protein 4
Localization
GO:0005634 nucleus 2 GO:0005739 mitochondrion 1
Pathway
R-HSA-392499 Metabolism of proteins 2 R-HSA-1430728 Metabolism 1 R-HSA-4839726 Chromatin organization 1 R-HSA-8953854 Metabolism of RNA 1
Partners
Complex memberships
HEMK2–TRMT112 (KMT9) heterodimer

Evidence

Reading pass · 13 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2003 PrmC/HemK crystal structure resolved at 2.2 Å; the C-terminal domain adopts a canonical AdoMet-dependent methyltransferase fold; the conserved NPPY motif positions the glutamine substrate via hydrogen bonds orienting the nitrogen lone pair toward the AdoMet methyl group, establishing the N5-glutamine methylation catalytic mechanism. X-ray crystallography with substrate and product complexes; active-site structural analysis Biochemistry High 12741815
2005 E. coli PrmC methylates the glutamine residue in the GGQ motif of release factor RF1; crystal structure of the RF1–PrmC–AdoHCy complex shows both domain 3 (GGQ domain) and central domains 2/4 of RF1 contact PrmC; methylation stimulates peptide chain release activity of RF1. X-ray crystallography of RF1–PrmC complex; methylation assays; site-directed mutagenesis Molecular cell High 16364916
2005 Chlamydia trachomatis PrmC functions as an N5-glutamine AdoMet-dependent methyltransferase that methylates class I release factors at the GGQ motif; chlamydial PrmC can complement an E. coli prmC knockout in vivo. Complementation assay in E. coli prmC knockout; in vivo and in vitro methylation assay with recombinant proteins; tryptic fragment analysis Journal of bacteriology Medium 15629922
2006 Recombinant murine PRED28 (HEMK2) protein localizes to the nucleus but shows no detectable adenine DNA methyltransferase activity; N6-methyladenine is essentially absent from mammalian DNA (fewer than 10³ m6A per mouse genome). Subcellular localization by immunofluorescence/fractionation; in vitro methyltransferase activity assay; sensitive mass spectrometry detection of m6A in genomic DNA FEBS letters Medium 16684535
2008 Human HEMK2 (with TRMT112 as partner) methylates human and yeast eRF1 in complex with eRF3 and GTP in vitro; the human HEMK2 catalytic subunit complements growth defect of yeast mtq2 deletion strains, confirming conserved eRF1 glutamine methyltransferase function. In vitro methylation assay with recombinant human HEMK2–TRMT112; yeast complementation of mtq2Δ FEBS letters High 18539146
2016 Murine HEMK2 requires a GQX3R motif for glutamine methylation activity; HEMK2 methylates the Gln185 residue of eRF1 and at least 11 additional human protein domains in vitro; CHD5 and NUT are methylated by HEMK2 in HEK293 cells. Peptide SPOT array specificity profiling; in vitro methylation of recombinant protein domains; in-cell methylation assay with transfected substrates The Journal of biological chemistry High 26797129
2019 Human HEMK2 (C21orf127), designated KMT9α, forms an obligate heterodimer with TRMT112 (KMT9β) and monomethylates lysine 12 of histone H4 (H4K12me1) in vitro and in vivo; crystal structure of KMT9 with SAH and H4K12me1 peptide reveals the structural basis for H4K12 recognition; KMT9 enriches at promoters of cell cycle regulator genes and is required for prostate cancer cell proliferation. In vitro histone methyltransferase assay; X-ray crystallography of KMT9–SAH–H4K12me1 peptide complex; ChIP-seq; siRNA knockdown with proliferation and cell cycle assays; xenograft mouse model Nature structural & molecular biology High 31061526
2020 Crystal structures of HEMK2–TRMT112 bound to SAM and to SAH with methylglutamine reveal a specific pocket in HEMK2 that accommodates the substrate glutamine and catalyzes its methylation; mass spectrometry confirms eRF1 glutamine methylation, demonstrating dual (Gln and Lys) methyltransferase activity of HEMK2. X-ray crystallography (two structures); mass spectrometry-based methylation verification The Biochemical journal High 32969463
2021 A biotinylated NTMT1 bisubstrate inhibitor (NAH-C3-GPKK analogue) pulls down the endogenous HemK2–TRMT112 complex from cell lysates, and the parent compound NAH-C3-GPKK potently inhibits HemK2–TRMT112 methyltransferase activity, representing the first reported potent inhibitor of this complex. Chemoproteomic pulldown with biotinylated probe; competitive biochemical inhibition assay ACS chemical biology Medium 34192867
2024 HEMK2 prefers glutamine over lysine methylation at both peptide and protein levels; the eRF1 sequence is strongly preferred over H4K12; Q-methylation prefers a G-Q-X3-R context while K-methylation prefers S/T at the first position; SETD6, not HEMK2, is the primary H4K12me1 methyltransferase in DU145 prostate cancer cells (HEMK2 activity ~1000-fold lower than SETD6 on H4K12). Peptide SPOT array methylation; in vitro protein methylation assays; siRNA knockdown combined with mass spectrometry quantification of H4K12me1 in DU145 cells Protein science Medium 38284488
2024 A bi-substrate KMT9 inhibitor (KMI169) targets both the SAM and substrate-binding pockets of KMT9, determined by structure-based drug design; KMI169 selectively downregulates KMT9 target genes involved in cell cycle regulation and impairs proliferation of castration- and enzalutamide-resistant prostate cancer cells. Structure-based drug design with co-crystal structures; biochemical selectivity profiling; cellular target engagement assays; gene expression analysis; proliferation assays Nature communications High 38167811
2024 In Drosophila, HemK2 methylates eRF1 in germline cells; knockdown of hemK2 reduces eRF1 methylation and protein synthesis, induces ribosomal stalling and disome formation, activates No-Go Decay leading to mRNA degradation, and causes apoptosis during oogenesis; overexpression of a methylation-deficient eRF1 recapitulates these defects, establishing eRF1 as the primary functional substrate. Germline-specific RNAi knockdown; methylation assays; polysome profiling (disome detection); No-Go Decay pathway inhibition rescue; genetic epistasis with methylation-deficient eRF1 overexpression Development (Cambridge, England) High 38881530
2025 KMT9 localizes to mitochondria of prostate cancer cells (but not other tumor cell types) and monomethylates DLAT (dihydrolipoamide transacetylase) at lysine 596; this methylation regulates pyruvate dehydrogenase complex (PDC) activity; KMT9 depletion reduces DLAT K596me1, impairs PDC activity and de novo lipogenesis, and inhibits prostate cancer cell proliferation in vitro and in vivo. Subcellular fractionation and mitochondrial localization assays; in vitro and in vivo methylation assays identifying DLAT K596; PDC activity assay; de novo lipogenesis measurement; siRNA knockdown; mouse xenograft model; patient tissue correlation Nature communications High 39885202

Source papers

Stage 0 corpus · 20 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2005 Molecular basis for bacterial class I release factor methylation by PrmC. Molecular cell 73 16364916
2008 HemK2 protein, encoded on human chromosome 21, methylates translation termination factor eRF1. FEBS letters 71 18539146
2019 KMT9 monomethylates histone H4 lysine 12 and controls proliferation of prostate cancer cells. Nature structural & molecular biology 68 31061526
2006 Undetectable levels of N6-methyl adenine in mouse DNA: Cloning and analysis of PRED28, a gene coding for a putative mammalian DNA adenine methyltransferase. FEBS letters 63 16684535
2003 Structures along the catalytic pathway of PrmC/HemK, an N5-glutamine AdoMet-dependent methyltransferase. Biochemistry 52 12741815
2020 Depletion of histone methyltransferase KMT9 inhibits lung cancer cell proliferation by inducing non-apoptotic cell death. Cancer cell international 33 32095117
2016 Substrate Specificity of the HEMK2 Protein Glutamine Methyltransferase and Identification of Novel Substrates. The Journal of biological chemistry 32 26797129
2021 KMT9 Controls Stemness and Growth of Colorectal Cancer. Cancer research 26 34737213
2012 The peptide chain release factor methyltransferase PrmC is essential for pathogenicity and environmental adaptation of Pseudomonas aeruginosa PA14. Environmental microbiology 23 23278968
2005 The N5-glutamine S-adenosyl-L-methionine-dependent methyltransferase PrmC/HemK in Chlamydia trachomatis methylates class 1 release factors. Journal of bacteriology 20 15629922
2021 Chemoproteomic Study Uncovers HemK2/KMT9 As a New Target for NTMT1 Bisubstrate Inhibitors. ACS chemical biology 9 34192867
2020 Structural insight into HEMK2-TRMT112-mediated glutamine methylation. The Biochemical journal 8 32969463
2024 Distinct specificities of the HEMK2 protein methyltransferase in methylation of glutamine and lysine residues. Protein science : a publication of the Protein Society 7 38284488
2016 Unravelling post-transcriptional PrmC-dependent regulatory mechanisms in Pseudomonas aeruginosa. Environmental microbiology 7 27376486
2025 Mitochondrial KMT9 methylates DLAT to control pyruvate dehydrogenase activity and prostate cancer growth. Nature communications 6 39885202
2024 Structure-guided design of a selective inhibitor of the methyltransferase KMT9 with cellular activity. Nature communications 6 38167811
2024 HemK2 functions for sufficient protein synthesis and RNA stability through eRF1 methylation during Drosophila oogenesis. Development (Cambridge, England) 5 38881530
2024 Lysine Methyltransferase 9 (KMT9) Is an Actionable Target in Muscle-Invasive Bladder Cancer. Cancers 3 38672614
2025 Structure-Guided Design of a KMT9 Inhibitor Prodrug with Cellular Activity. Journal of medicinal chemistry 0 40526927
2020 The Peptide Chain Release Factor Methyltransferase PrmC Influences the Pseudomonas aeruginosa PA14 Endo- and Exometabolome. Metabolites 0 33080992

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