Affinage

METTL18

Histidine protein methyltransferase 1 homolog · UniProt O95568

Length
372 aa
Mass
42.1 kDa
Annotated
2026-06-10
20 papers in source corpus 9 papers cited in narrative 9 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

METTL18 is a nuclear, nucleolus-enriched histidine-specific protein methyltransferase that stoichiometrically monomethylates the 60S ribosomal protein RPL3 to regulate translation elongation and proteostasis (PMID:33693809, PMID:35674491). It deposits a 3-methylhistidine (τ-N) mark on His-245 of RPL3, requires a functional nuclear localization signal, and is itself automethylated at His-154; the enzyme was identified as the most enriched methyltransferase in an RPL3 interactomics screen (PMID:33693809). The yeast ortholog Hpm1p establishes that this activity acts on ribosome-associated rather than free RPL3 substrate, indicating that methylation depends on the assembled ribosomal context (PMID:24865971). Functionally, the RPL3 modification slows ribosome traversal of Tyr (and Pro) codons, providing a kinetic window for proper folding of nascent proteins; loss of METTL18 accelerates elongation at these codons and causes aggregation of codon-enriched proteins (PMID:35674491, PMID:41713742). Consistent with a role in ribosome maturation, METTL18 loss impairs pre-rRNA processing and reduces polysome/60S formation (PMID:33693809, PMID:24865971), and separation-of-function analysis in yeast shows RPL3-His methylation specifically governs translation elongation fidelity rather than subunit biogenesis, implying additional substrates (PMID:26826131). In vivo, Mettl18 knockout mice develop diabetic phenotypes with accumulation of pancreatitis-associated proteins (e.g., Reg1) and unfolded protein response activation, tying the enzyme to organ-level proteostasis (PMID:41713742). A further role in HER2-negative breast cancer has been described in which METTL18 indirectly supports HSP90 integrity, actin polymerization, and Src phosphorylation to promote metastatic signaling (PMID:39309445).

Mechanistic history

Synthesis pass · year-by-year structured walk · 8 steps
  1. 2010 High

    Established the founding biochemical identity of the enzyme: the yeast ortholog Hpm1p is the methyltransferase responsible for stoichiometric 3-methylhistidine monomethylation of ribosomal protein Rpl3 at His-243.

    Evidence Top-down/bottom-up MS, in vivo SAM-[3H] radiolabeling, cation-exchange chromatography and deletion-strain screening of methyltransferase candidates in yeast

    PMID:20864530

    Open questions at the time
    • Did not identify the human ortholog or test mammalian activity
    • Catalytic mechanism and structural basis of histidine specificity not resolved
  2. 2014 High

    Defined the substrate requirement and cellular consequences: Hpm1p methylates ribosome-associated but not free Rpl3p, and its loss disrupts early rRNA processing, 60S subunit levels, and translational fidelity.

    Evidence In vitro methyltransferase assays with ribosome-associated vs. free substrate, polysome profiling, rRNA processing assays, drug-resistance and fidelity reporters in yeast

    PMID:24865971

    Open questions at the time
    • Whether biogenesis and fidelity defects arise from one or multiple substrates unresolved
    • No mechanistic link between methylation and elongation kinetics yet
  3. 2016 High

    Separated the enzyme's functions: blocking Rpl3 His-243 methylation alone phenocopies fidelity and rRNA processing defects but not subunit imbalance, implying Hpm1p acts on additional substrates.

    Evidence Rpl3-H243A separation-of-function mutagenesis with stop-codon readthrough, misincorporation, and frameshifting assays in yeast

    PMID:26826131

    Open questions at the time
    • Identity of the additional substrate(s) driving subunit-level effects unknown
    • Codon-level basis of fidelity change not defined
  4. 2021 High

    Transferred the mechanism to humans: METTL18 methylates His-245 of RPL3, localizes to nucleoli via an NLS, is automethylated, and its loss perturbs pre-rRNA processing and codon-specific translation.

    Evidence Recombinant in vitro methylation, RPL3 interactomics/Co-IP, MS detection of 3-methylhistidine, KO cell lines, polysome profiling, and imaging in human cells

    PMID:33693809

    Open questions at the time
    • Functional consequence of automethylation at His-154 unknown
    • Which translation defects are direct vs. secondary to rRNA processing not separated
  5. 2022 High

    Provided the kinetic-proteostasis mechanism: RPL3 τ-methylation slows ribosome traversal of Tyr codons to permit correct folding, and its loss accelerates Tyr-codon elongation and causes aggregation of Tyr-rich proteins.

    Evidence In vitro methylation with quantitative MS, cryo-EM of modified vs. unmodified ribosomes, genome-wide ribosome profiling, in vitro translation, and aggregation assays in METTL18 KO cells

    PMID:35674491

    Open questions at the time
    • Structural mechanism by which His methylation alters elongation rate not fully resolved
    • Scope of affected codons beyond Tyr in human cells limited
  6. 2022 Medium

    Broadened the downstream footprint: loss of Hpm1p alters protein conformations and interactions across ribosome, chromatin, membrane, and mitochondrial proteins independent of abundance, and changes stress sensitivity and metabolic protein levels.

    Evidence Quantitative crosslinking MS (XL-MS) with stable isotope labeling, targeted methylation stoichiometry MS, and growth assays in yeast

    PMID:35609787

    Open questions at the time
    • Causality between the single methyl mark and distal proteome changes not established
    • Direct vs. indirect interaction changes not distinguished
  7. 2024 Medium

    Implicated METTL18 in oncogenic signaling: its RPL3 methylation activity indirectly supports HSP90 integrity, actin polymerization, and Src phosphorylation to drive metastatic responses in HER2-negative breast cancer.

    Evidence Knockdown/overexpression in breast tumor lines, xenograft model, F/G-actin assays, and Western blot for Src and HSP90 in cells and in vivo

    PMID:39309445

    Open questions at the time
    • Mechanistic chain from RPL3 methylation to HSP90 is indirect and not reconstituted
    • Single-lab finding without orthogonal confirmation
  8. 2026 High

    Demonstrated organ-level physiological requirement: Mettl18 knockout mice develop diabetic phenotypes with pancreatitis-associated protein accumulation and UPR activation, driven by accelerated proline-codon elongation.

    Evidence Mettl18 knockout mouse model, ribosome profiling in pancreatic acinar cells, MS for N3-histidine methylation, aggregation assays, and UPR markers

    PMID:41713742

    Open questions at the time
    • Tissue specificity of the pancreatic phenotype not explained
    • Relationship between Tyr- and Pro-codon effects across tissues unresolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • The full substrate repertoire of METTL18 beyond RPL3 and the structural basis by which a single histidine methyl mark tunes codon-specific elongation remain open.
  • Additional substrates implied by yeast separation-of-function data not identified in mammals
  • No structural model of how the modification mechanistically slows elongation at specific codons
  • Direct link between the methylation activity and the breast-cancer HSP90/Src axis not reconstituted

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 3
Localization
GO:0005634 nucleus 1 GO:0005730 nucleolus 1
Pathway
R-HSA-8953854 Metabolism of RNA 3 R-HSA-392499 Metabolism of proteins 2
Partners

Evidence

Reading pass · 9 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2021 Human METTL18 is a histidine-specific methyltransferase that methylates His-245 of RPL3 (ribosomal protein L3), producing a 3-methylhistidine (τ-methylhistidine) modification. METTL18 localizes to the nucleus and accumulates in nucleoli, requires a functional nuclear localization signal, and was identified as the most significantly enriched MTase in an RPL3 interactomics screen. METTL18 is also automethylated at its own His-154. METTL18 knockout cells show altered pre-rRNA processing, decreased polysome formation, and codon-specific changes in mRNA translation. Recombinant protein in vitro methylation assay, RPL3 interactomics/Co-IP screen, mass spectrometry identification of 3-methylhistidine, METTL18 KO cell lines, polysome profiling, nuclear/nucleolar localization by imaging Nucleic acids research High 33693809
2022 METTL18 methylates His-245 of RPL3 at the τ-N position in vitro and in cells. This modification specifically slows ribosome traversal on Tyr codons, allowing proper folding of newly synthesized proteins. Ribosome profiling showed that loss of METTL18 accelerates translation elongation at Tyr codons, and RPL3 methylation protects cells from aggregation of Tyr-rich proteins, linking histidine methylation to proteostasis maintenance. In vitro methylation assay with methyl-donor analog and quantitative MS, cryo-EM structural comparison of modified vs. unmodified ribosomes, genome-wide ribosome profiling, in vitro translation assay, protein aggregation assay in METTL18 KO cells eLife High 35674491
2024 METTL18-mediated methylation of RPL3 indirectly regulates HSP90 integrity and protein levels, promoting actin polymerization via HSP90, which in turn leads to Src phosphorylation at Tyr-419 and Tyr-530 and downstream oncogenic signaling in HER2-negative breast cancer cells. Loss of METTL18 reduces metastatic responses in vitro and in vivo. METTL18 knockdown/overexpression in breast tumor cell lines, tumor xenograft model, confocal microscopy, F/G-actin assays, Western blot for Src phosphorylation and HSP90 International journal of biological sciences Medium 39309445
2026 METTL18 is essential for pancreatic function in vivo: Mettl18 knockout mice show diabetic phenotypes, accumulation of pancreatitis-associated proteins, and activation of the unfolded protein response. Ribosome profiling in pancreatic acinar cells revealed that loss of METTL18 causes global translational alterations including accelerated elongation at proline codons, leading to improper protein folding and aggregation of pancreatitis-associated proteins (e.g., Reg1). Mettl18 knockout mouse model, ribosome profiling in pancreatic acinar cell line, mass spectrometry for N3-histidine methylation, protein aggregation assays, unfolded protein response markers Molecular metabolism High 41713742
2010 Yeast Hpm1p (ortholog of human METTL18; encoded by YIL110W, also designated HPM1) is a seven-β-strand methyltransferase responsible for stoichiometric monomethylation at His-243 of ribosomal protein Rpl3, producing 3-methylhistidine. Deletion of HPM1 abolishes this modification; the modification is found in ribosomes and nucleus-containing fractions but not in ribosome-free cytosol. Top-down and bottom-up mass spectrometry, in vivo radiolabeling with SAM-[methyl-3H], high-resolution cation-exchange chromatography, TLC, deletion strain analysis of 37 methyltransferase candidates The Journal of biological chemistry High 20864530
2014 Yeast Hpm1p (ortholog of human METTL18) has methyltransferase activity in vitro on ribosome-associated Rpl3p but NOT on free Rpl3p, indicating its activity depends on interactions with ribosomal components. hpm1-null cells show defective early rRNA processing, deficiency of 60S subunits, translation initiation defects, resistance to cycloheximide and verrucarin A, and decreased translational fidelity. In vitro methyltransferase assay with ribosome-associated vs. free Rpl3p, amino acid analysis, polysome profiling, rRNA processing assays, drug resistance plate assays, translational fidelity reporters Molecular and cellular biology High 24865971
2016 Methylation of Rpl3p at His-243 by Hpm1p (yeast ortholog of METTL18) plays a significant role in translation elongation fidelity. The rpl3-H243A mutation (blocking methylation at this site) phenocopies Hpm1-deficient cells in pre-rRNA processing defects and translational accuracy defects, but NOT in perturbed ribosomal subunit levels. This indicates Hpm1p has multiple substrates, with Rpl3p methylation specifically contributing to translation elongation fidelity rather than subunit biogenesis. Rpl3-H243A mutagenesis, translational fidelity assays (stop codon readthrough, amino acid misincorporation, -1 frameshifting), pre-rRNA processing analysis, ribosomal subunit profiling RNA (New York, N.Y.) High 26826131
2023 METTL18 is identified as one of the catalytic enzymes responsible for Nτ-methylhistidine modifications in mammals. A methodology using biochemical protein fractionation combined with LC-MS/MS quantification of methylhistidine was established; METTL18 is listed alongside SETD3 and METTL9 as known mammalian histidine methyltransferases. Biochemical fractionation, LC-MS/MS quantification of methylhistidine, in silico structural prediction Journal of biochemistry Low 37279646
2022 Loss of yeast Hpm1p (ortholog of human METTL18) results in changes to protein structure and protein-protein interactions in the ribosome, membrane proteins, chromatin, and mitochondria as detected by quantitative crosslinking MS, independently of changes in protein abundance. Hpm1p deletion also results in increased sensitivity to nonribosomal stressors and differential abundance of proteins linked to sugar metabolism coordination. Quantitative proteomics, stable isotope labeling crosslinking MS (XL-MS), targeted MS for H243 methylation stoichiometry, growth assays Molecular & cellular proteomics : MCP Medium 35609787

Source papers

Stage 0 corpus · 20 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2010 A novel 3-methylhistidine modification of yeast ribosomal protein Rpl3 is dependent upon the YIL110W methyltransferase. The Journal of biological chemistry 73 20864530
2021 Human METTL18 is a histidine-specific methyltransferase that targets RPL3 and affects ribosome biogenesis and function. Nucleic acids research 50 33693809
1997 New xenograft model of multiple myeloma and efficacy of a humanized antibody against human interleukin-6 receptor. Blood 46 9310495
2020 Serum proteome profiles revealed dysregulated proteins and mechanisms associated with fibromyalgia syndrome in women. Scientific reports 39 32704114
2022 METTL18-mediated histidine methylation of RPL3 modulates translation elongation for proteostasis maintenance. eLife 34 35674491
2020 Protein Histidine Methylation. Current protein & peptide science 32 32188384
2014 Histidine methylation of yeast ribosomal protein Rpl3p is required for proper 60S subunit assembly. Molecular and cellular biology 32 24865971
2016 Methylation of yeast ribosomal protein Rpl3 promotes translational elongation fidelity. RNA (New York, N.Y.) 29 26826131
2013 Two novel susceptibility SNPs for ischemic stroke using exome sequencing in Chinese Han population. Molecular neurobiology 17 24122314
2003 Humanized anti-interleukin-6 receptor monoclonal antibody induced apoptosis of fresh and cloned human myeloma cells in vitro. Leukemia research 17 12531226
2016 Ribosomal protein methyltransferases in the yeast Saccharomyces cerevisiae: Roles in ribosome biogenesis and translation. Biochemical and biophysical research communications 14 26801560
1998 IL-6 functions in cynomolgus monkeys blocked by a humanized antibody to human IL-6 receptor. International journal of immunopharmacology 13 9756130
1996 Therapeutic potential of humanized anti-interleukin-6 receptor antibody: antitumor activity in xenograft model of multiple myeloma. Anticancer research 13 8917348
1997 Safety and kinetic properties of a humanized antibody to human interleukin-6 receptor in healthy non-human primates. Toxicology 5 9328216
1997 Identification of the metabolites of a new oxazolidinone MAO-A inhibitor in rat. Xenobiotica; the fate of foreign compounds in biological systems 5 9364743
2025 Identification of novel small molecule inhibitors targeting multiple methyltransferase like proteins against hepatocellular carcinoma. Scientific reports 4 41006418
2024 METTL18 functions as a Phenotypic Regulator in Src-Dependent Oncogenic Responses of HER2-Negative Breast Cancer. International journal of biological sciences 2 39309445
2023 γ-enolase (ENO2) is methylated at the Nτ position of His-190 among enolase isozymes. Journal of biochemistry 2 37279646
2026 METTL18 ensures pancreatic function by maintaining proper translation and proteostasis. Molecular metabolism 0 41713742
2022 Differential Proteome and Interactome Analysis Reveal the Basis of Pleiotropy Associated With the Histidine Methyltransferase Hpm1p. Molecular & cellular proteomics : MCP 0 35609787

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