Affinage

MAML2

Mastermind-like protein 2 · UniProt Q8IZL2

Length
1156 aa
Mass
125.2 kDa
Annotated
2026-06-10
100 papers in source corpus 16 papers cited in narrative 16 extracted findings
Cross-family judge vs UniProt: tie faithfulness: 5/5 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

MAML2 is a transcriptional coactivator that bridges DNA-bound transcription factors to the CBP/p300 acetyltransferase machinery: its C-terminal transactivation domain 1 (TAD1) binds directly to the CBP KIX domain through an ~20-residue segment that contacts the same KIX surface used by MLL1, providing the molecular basis for coactivator recruitment (PMID:26274502). In its native context the N-terminal basic domain of MAML2 binds the Notch intracellular domain to support Notch target gene transcription, and loss or dominant-negative disruption of this function impairs Notch signaling—silencing MAML2 downregulates Notch targets (PMID:27207652), and a pathogenic MAML2 variant acts dominant-negatively to block HES1-dependent thyroid hormone biosynthesis genes, linking MAML2 to congenital hypothyroidism through Notch control of thyroid cells (PMID:36898841). MAML2's dominant disease relevance is as the 3' partner in recurrent oncogenic fusions. In the CRTC1(MECT1)-MAML2 fusion of mucoepidermoid carcinoma, the MAML2 N-terminal Notch-binding domain is replaced by the CRTC1 N-terminus, which tethers CREB and recruits p300/CBP to constitutively activate CREB-dependent transcription (PMID:15961999, PMID:14720503); this transforming activity depends on an intact CREB-binding domain (PMID:16103063) and on sustained fusion expression, which is required for tumor growth and is a causal, 100%-penetrant driver of salivary gland tumors in mice (PMID:16652146, PMID:33830080). The fusion drives a network of mitogenic programs—autocrine AREG/EGFR signaling (PMID:23975434), a PGC-1α4/PPARγ/IGF-1 circuit (PMID:33626346), MYC target gene activation through direct MYC interaction (PMID:25071166), and CREB-dependent induction of the lncRNA LINC00473, which binds NONO to reinforce CREB-mediated transcription (PMID:29353885). In a parallel oncogenic context, YAP1-MAML2 fusions act through Hippo-resistant, TEAD-dependent YAP transcriptional activity to drive poromas and meningioma-like tumors (PMID:31145701, PMID:36008139), and form phase-separated condensates that selectively amplify a subset of YAP target genes including CTGF and CYR61 (PMID:38315854).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 2004 Medium

    Established that the CRTC1(WAMTP1)-MAML2 fusion swaps out the MAML2 N-terminal basic domain needed for Notch ICD binding, repositioning the fusion away from native Notch coactivation.

    Evidence Fusion cloning, domain deletion/mutation analysis, immunofluorescence co-localization, and Notch target gene expression in primary MEC tumors

    PMID:14720503

    Open questions at the time
    • Did not establish the gain-of-function transcriptional mechanism of the fusion
    • Altered Notch targets observed only correlatively in tumor samples
  2. 2005 High

    Defined the gain-of-function mechanism: the fusion tethers CREB and recruits p300/CBP to constitutively activate CREB target genes, and showed CREB DNA binding is required for transformation.

    Evidence Co-IP, reporter assays, dominant-negative CREB blocking, and epithelial transformation assays; reinforced by in-frame deletion of the CREB-binding domain abolishing transforming activity in RK3E cells

    PMID:15961999 PMID:16103063

    Open questions at the time
    • Did not map the precise MAML2 surface recruiting p300/CBP
    • Notch target genes unchanged, leaving native MAML2 contribution unaddressed
  3. 2006 High

    Showed that sustained fusion expression is continuously required for tumor cell growth, establishing oncogene dependence.

    Evidence Hairpin RNAi knockdown, colony formation, RNAi-resistant rescue, and xenograft assays in t(11;19) MEC lines

    PMID:16652146

    Open questions at the time
    • Did not identify the downstream effectors mediating dependence
  4. 2007 Medium

    Showed that a distinct MLL-MAML2 fusion also deletes the Notch-binding domain and acts dominant-negatively on Notch target transcription.

    Evidence Breakpoint RT-PCR/sequencing and luciferase reporter for NOTCH1-ICD-driven HES1 activity

    PMID:17551948

    Open questions at the time
    • Limited to a reporter readout without cellular transformation data
    • Biological consequence in primary tumors not established
  5. 2013 High

    Identified autocrine AREG/EGFR signaling as a druggable downstream output of the CRTC1-MAML2/CREB axis.

    Evidence Expression analysis, fusion knockdown, pharmacological EGFR inhibition, and MEC xenografts

    PMID:23975434

    Open questions at the time
    • Did not establish whether EGFR signaling alone accounts for growth dependence
  6. 2014 Medium

    Revealed a gain-of-function interaction with MYC, expanding the fusion's transcriptional program to MYC targets required for transformation.

    Evidence Co-IP of the fusion with MYC, expression profiling, and transformation assays in endogenous-fusion MEC cells

    PMID:25071166

    Open questions at the time
    • Single-lab Co-IP without reciprocal structural mapping of the interaction
    • Direct vs indirect nature of the MYC interaction not resolved
  7. 2015 Medium

    Provided the molecular basis for coactivator recruitment by mapping direct MAML2 TAD1 binding to the CBP KIX domain at the MLL1-binding surface.

    Evidence In vitro peptide binding (NMR/biochemical) and sequence/structural analysis of the KIX interface

    PMID:26274502

    Open questions at the time
    • No mutagenesis validation of the interface in a cellular context
    • Affinity measured for an isolated peptide, not full-length fusion
  8. 2018 Medium

    Showed CREB-dependent induction of the lncRNA LINC00473, which binds NONO to amplify fusion-driven CREB transcription and supports tumor growth.

    Evidence Expression profiling, siRNA knockdown, xenografts, RNA-ISH, and RNA pull-down for NONO

    PMID:29353885

    Open questions at the time
    • Single lab; mechanism of NONO-mediated transcriptional enhancement incompletely defined
  9. 2021 High

    Established CRTC1-MAML2 as a causal, fully penetrant in vivo driver and uncovered cooperating p16-CDK4/6-RB pathway alterations and PGC-1α4/PPARγ/IGF-1 circuitry as combination-therapy targets.

    Evidence Conditional transgenic mouse model, dox-inducible shRNA in xenografts, pathway analysis, and combined EGFR/CDK4/6 or IGF-1R/PPARγ pharmacology

    PMID:33626346 PMID:33830080

    Open questions at the time
    • Relative contribution of the parallel mitogenic circuits to tumor maintenance not ranked
    • PGC-1α4/PPARγ/IGF-1 findings from a single lab
  10. 2022 High

    Demonstrated that YAP1-MAML2 fusions drive tumors via Hippo-resistant, TEAD-dependent YAP activity, with the YAP component being the critical oncogenic driver.

    Evidence In vivo mouse expression model, expression profiling vs NF2-mutant meningiomas, constitutively active YAP1 mutagenesis, and YAP-TEAD inhibitor treatment ex vivo; building on RNA-seq/reporter identification of the fusion in poromas

    PMID:31145701 PMID:36008139

    Open questions at the time
    • Functional contribution of the MAML2 portion to the YAP fusion not isolated
    • Mechanism of Hippo resistance not structurally defined
  11. 2023 Medium

    Linked native MAML2 loss-of-function to human disease, showing a dominant-negative variant blocks HES1-dependent thyroid hormone biosynthesis and causes congenital hypothyroidism.

    Evidence Variant identification, reporter/functional assays in HEK293T and thyroid cells, thyroid organoids, and zebrafish/mouse Notch inhibition models

    PMID:36898841

    Open questions at the time
    • Based on a single patient variant
    • Generalizability to other MAML2 loss-of-function alleles untested
  12. 2024 Medium

    Resolved how YAP1-MAML2 organizes transcription, showing phase-separated condensates selectively amplify a small subset of target genes rather than the bulk program.

    Evidence Live-cell phase-separation imaging, chemogenetic condensate dissolution at matched protein levels, and paired transcriptome comparison

    PMID:38315854

    Open questions at the time
    • Single lab with a novel chemogenetic tool
    • Mechanism selecting which genes require condensates is undefined

Open questions

Synthesis pass · forward-looking unresolved questions
  • How native full-length MAML2 coactivation is regulated and integrated across distinct transcription factor partners (Notch, CREB, TEAD) in non-fusion physiological contexts remains incompletely defined.
  • No structural model of full-length MAML2 in any native complex
  • Endogenous MAML2 function in normal tissues largely uncharacterized in the corpus

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140110 transcription regulator activity 4 GO:0060090 molecular adaptor activity 2
Localization
GO:0005634 nucleus 2 GO:0005654 nucleoplasm 1
Pathway
R-HSA-162582 Signal Transduction 4 R-HSA-1643685 Disease 4 R-HSA-74160 Gene expression (Transcription) 3 R-HSA-4839726 Chromatin organization 1
Partners
CREB1CREBBPEP300MYCNONONOTCH1TEAD
Complex memberships
CREB transcriptional complexTEAD transcriptional complex

Evidence

Reading pass · 16 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2005 The MECT1-MAML2 (CRTC1-MAML2) fusion protein binds to CREB and recruits p300/CBP into the CREB transcriptional complex through a binding domain on the MAML2 portion, constitutively activating CREB-dependent transcription and inducing expression of multiple CREB target genes. The transforming activity of MECT1-MAML2 was markedly reduced by blocking CREB DNA binding. Co-immunoprecipitation, reporter assays, gene expression analysis, dominant-negative CREB blocking, transformation assays in epithelial cells The EMBO journal High 15961999
2005 The transforming activity of the MECT1-MAML2 fusion oncogene depends on an intact CREB-binding domain derived from the MECT1/TORC1 N-terminus; small in-frame deletions within this CREB-binding domain completely abolished transforming activity in RK3E epithelial cells. The fusion strongly activated cAMP/CREB-regulated genes but did not alter known Notch-regulated target genes in expression profiling experiments. In-frame deletion mutagenesis of the CREB-binding domain, transformation assay in RK3E cells, doxycycline-regulated expression system, global gene expression profiling, RT-PCR validation Cancer research High 16103063
2006 Sustained expression of the MECT1-MAML2 fusion is required for tumor cell growth: RNAi-mediated knockdown of the fusion peptide caused ≥90% colony growth inhibition in MEC tumor cell lines carrying the t(11;19) rearrangement. Re-expression of an RNAi-resistant mutant MECT1-MAML2 partially rescued growth inhibition, confirming on-target specificity. Hairpin RNAi knockdown, colony formation assay, rescue experiment with RNAi-resistant mutant, in vivo xenograft in nude mice Oncogene High 16652146
2004 The WAMTP1(CRTC1)-MAML2 fusion protein loses the N-terminal basic domain of MAML2 required for binding to intracellular Notch (Notch ICD). Mutation analysis identified two regions in the WAMTP1 N-terminus important for nuclear localization (amino acids 11-20) and for co-localization with MAML2 and Notch1 ICD in nuclear granules (amino acids 21-42). Analysis of Notch target genes in fusion-positive vs. negative MEC tumors showed upregulation of HES5 and downregulation of MASH1, indicating altered Notch signaling. Fusion gene cloning, domain deletion/mutation analysis, immunofluorescence co-localization, Notch target gene expression analysis in primary tumor samples Experimental cell research Medium 14720503
2013 The CRTC1-MAML2 fusion oncoprotein co-activates CREB transcription factor to induce upregulation of the EGFR ligand Amphiregulin (AREG), which then activates EGFR signaling in an autocrine manner promoting MEC cell growth and survival. CRTC1-MAML2-positive MEC cells were highly sensitive to EGFR signaling inhibition in vitro and in vivo. Gene expression analysis, RNA interference knockdown of CRTC1-MAML2, pharmacological EGFR inhibition, human MEC xenograft models in vivo Oncogene High 23975434
2014 The CRTC1-MAML2 (C1/M2) oncoprotein interacts with MYC proteins and activates MYC transcription targets involved in cell growth, metabolism, survival, and tumorigenesis. This gain-of-function interaction with MYC is necessary for C1/M2-driven cell transformation. Co-immunoprecipitation of C1/M2 with MYC, gene expression profiling, transformation assay, validation in human MEC tumor cells harboring the t(11;19) translocation Proceedings of the National Academy of Sciences of the United States of America Medium 25071166
2015 A peptide derived from transactivation domain 1 (TAD1) of MAML2 binds directly to the CBP KIX domain with micromolar affinity. An ~20-residue segment within TAD1, conserved in MAML2 orthologs and paralogs, contacts a KIX surface previously shown to bind MLL1, sharing sequence and structural similarity with MLL1 at those contact positions. This provides the molecular basis for constitutive CBP/p300 recruitment by CRTC1/3-MAML2 fusion proteins. In vitro peptide binding assay (NMR/biochemical), sequence and structural analysis of KIX-binding interface Biochemistry Medium 26274502
2021 CRTC1-MAML2 is a causal oncogenic driver for MEC: doxycycline-induced knockdown blocked growth of established MEC xenografts; conditional transgenic Cre-induced CRTC1-MAML2 expression caused 100% penetrant salivary gland tumor formation in mice resembling human MEC histologically and molecularly. Molecular analysis revealed altered p16-CDK4/6-RB pathway activity as a cooperating event; co-targeting AREG/EGFR signaling (Erlotinib) and CDK4/6 (Palbociclib) produced enhanced anti-tumor responses in vitro and in vivo. Doxycycline-inducible shRNA knockdown in xenografts, conditional transgenic mouse model (Cre-induced expression), molecular pathway analysis, pharmacological combination therapy in vitro and in vivo JCI insight High 33830080
2021 CRTC1-MAML2 induces transcriptional activation of the non-canonical PGC-1α splice variant PGC-1α4, which regulates PPARγ-mediated IGF-1 expression, creating an autocrine mitogenic circuit. C1/M2-positive tumor cells are selectively sensitive to IGF-1R inhibition and to PPARγ inverse agonists. Gene expression profiling, small-molecule drug screens, knockdown/overexpression of pathway components, pharmacological inhibition of IGF-1R and PPARγ in MEC cell lines and primary tumors Cell reports Medium 33626346
2018 CRTC1-MAML2 fusion-induced transcription of LINC00473 (a lncRNA) is dependent on the ability of CRTC1-MAML2 to activate CREB-mediated transcription. LINC00473 depletion reduced MEC cell proliferation and survival in vitro and blocked tumor growth in xenografts. LINC00473 binds the cAMP signaling component NONO, enhancing CRTC1-MAML2-driven CREB-mediated transcription. Gene expression profiling, siRNA knockdown, in vivo xenograft, RNA in situ hybridization, RNA pull-down assay for NONO binding Oncogene Medium 29353885
2007 The MLL-MAML2 fusion, created by inv(11)(q21q23), encodes a chimeric protein in which the N-terminal basic domain of MAML2 (required for binding Notch ICD) is deleted. Luciferase assay demonstrated that MLL-MAML2 suppresses HES1 promoter activation by the NOTCH1 intracellular domain, indicating disruption of Notch signaling. RT-PCR and sequencing of fusion breakpoints, luciferase reporter assay for NOTCH1-ICD-driven HES1 promoter activity Genes, chromosomes & cancer Medium 17551948
2019 YAP1-MAML2 fusion proteins (identified by RNA-seq in poromas and porocarcinomas) strongly transactivate a TEAD reporter and promote anchorage-independent growth, confirming tumorigenic activity driven by YAP1/TEAD-dependent transcription. The YAP1 N-terminus (retained in the fusion) directs nuclear localization detectable by immunohistochemistry, while the YAP1 C-terminus is lost. RNA sequencing, RT-PCR, FISH confirmation of genomic rearrangements, TEAD luciferase reporter assay, anchorage-independent growth assay, immunohistochemistry The Journal of clinical investigation High 31145701
2022 YAP1-MAML2 fusion primarily functions by exerting TEAD-dependent YAP activity that is resistant to Hippo signaling. Expression of YAP1-MAML2 in mice induces meningioma-like tumors; constitutively active YAP1 (S127/397A) induces similar tumors, indicating that the YAP component is the critical oncogenic driver. Treatment with YAP-TEAD inhibitors inhibited viability of YAP1-MAML2-driven mouse tumors ex vivo. In vivo mouse expression model, gene expression profiling comparing YAP1 fusion-positive vs NF2 mutant meningiomas, YAP-TEAD inhibitor treatment ex vivo, constitutively active YAP1 mutagenesis Genes & development High 36008139
2024 YAP1-MAML2 fusion undergoes phase separation (PS) and forms liquid-like condensates with hallmarks of transcriptional activity. Using a chemogenetic tool to dissolve condensates at identical protein levels, PS was found to selectively further amplify expression of a small fraction of YAP1-MAML2 target genes (including CTGF and CYR61), while the majority of YAP1-MAML2-regulated genes are not affected by PS and can be activated by diffuse TF complexes. Live-cell phase separation imaging, chemogenetic condensate dissolution tool, comparative transcriptome analysis under phase-separated vs. non-phase-separated conditions Proceedings of the National Academy of Sciences of the United States of America Medium 38315854
2016 Stilbenoids (resveratrol and pterostilbene) induce de novo methylation at the MAML2 enhancer region, leading to transcriptional silencing of MAML2. This coincides with increased occupancy of repressive histone marks, decreased activating marks, binding of DNMT3B, and decreased occupancy of OCT1 transcription factor at the MAML2 enhancer, resulting in downregulation of NOTCH target genes in breast cancer cells. Genome-wide Illumina 450K DNA methylation array, ChIP for histone marks and DNMT3B/OCT1, gene expression analysis Carcinogenesis Medium 27207652
2023 A pathogenic MAML2 variant identified in a patient with congenital hypothyroidism exerts a dominant-negative effect on canonical Notch signaling and on thyroid hormone biosynthesis gene expression. In vitro functional assays in HEK293T and thyroid cells, organoid culture, and zebrafish/mouse models showed that Notch signaling within thyroid cells directly affects thyroid hormone biosynthesis; the MAML2 variant blocked HES1-dependent hormone biosynthesis gene expression. NGS variant identification, in vitro reporter/functional assays in HEK293T and Nthy-ori 3.1 cells, primary mouse thyroid organoid culture with transcriptome sequencing, zebrafish and mouse Notch inhibition models Journal of medical genetics Medium 36898841

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2010 A reappraisal of the MECT1/MAML2 translocation in salivary mucoepidermoid carcinomas. The American journal of surgical pathology 212 20588178
2019 Recurrent YAP1-MAML2 and YAP1-NUTM1 fusions in poroma and porocarcinoma. The Journal of clinical investigation 201 31145701
2006 MECT1-MAML2 fusion transcript defines a favorable subset of mucoepidermoid carcinoma. Clinical cancer research : an official journal of the American Association for Cancer Research 171 16818685
2009 Clinicopathological significance of the CRTC3-MAML2 fusion transcript in mucoepidermoid carcinoma. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 134 19749740
2005 Transforming activity of MECT1-MAML2 fusion oncoprotein is mediated by constitutive CREB activation. The EMBO journal 123 15961999
2004 Altered Notch signaling resulting from expression of a WAMTP1-MAML2 gene fusion in mucoepidermoid carcinomas and benign Warthin's tumors. Experimental cell research 118 14720503
2005 Mect1-Maml2 fusion oncogene linked to the aberrant activation of cyclic AMP/CREB regulated genes. Cancer research 114 16103063
2004 A study of MECT1-MAML2 in mucoepidermoid carcinoma and Warthin's tumor of salivary glands. The Journal of molecular diagnostics : JMD 103 15269296
2018 MAML2 Rearrangements in Variant Forms of Mucoepidermoid Carcinoma: Ancillary Diagnostic Testing for the Ciliated and Warthin-like Variants. The American journal of surgical pathology 92 28877061
2008 A new type of MAML2 fusion in mucoepidermoid carcinoma. Genes, chromosomes & cancer 87 18050304
2007 Frequent fusion of the CRTC1 and MAML2 genes in clear cell variants of cutaneous hidradenomas. Genes, chromosomes & cancer 82 17334997
2016 Stilbenoids remodel the DNA methylation patterns in breast cancer cells and inhibit oncogenic NOTCH signaling through epigenetic regulation of MAML2 transcriptional activity. Carcinogenesis 81 27207652
2011 Fluorescence in situ hybridization for detection of MAML2 rearrangements in oncocytic mucoepidermoid carcinomas: utility as a diagnostic test. Human pathology 81 21777943
2005 Clear cell hidradenoma of the skin-a third tumor type with a t(11;19)--associated TORC1-MAML2 gene fusion. Genes, chromosomes & cancer 79 15729701
2008 t(11;19) translocation and CRTC1-MAML2 fusion oncogene in mucoepidermoid carcinoma. Oral oncology 77 18486532
2013 Aberrantly activated AREG-EGFR signaling is required for the growth and survival of CRTC1-MAML2 fusion-positive mucoepidermoid carcinoma cells. Oncogene 74 23975434
2006 Sustained expression of Mect1-Maml2 is essential for tumor cell growth in salivary gland cancers carrying the t(11;19) translocation. Oncogene 65 16652146
2019 Metaplastic thymoma: a distinctive thymic neoplasm characterized by YAP1-MAML2 gene fusions. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 63 31641231
2017 Correlation of Crtc1/3-Maml2 fusion status, grade and survival in mucoepidermoid carcinoma. Oral oncology 63 28438292
2020 Recurrent YAP1 and MAML2 Gene Rearrangements in Retiform and Composite Hemangioendothelioma. The American journal of surgical pathology 62 32991341
2021 The CRTC1-MAML2 fusion is the major oncogenic driver in mucoepidermoid carcinoma. JCI insight 60 33830080
2012 Clinicopathological significance of MAML2 gene split in mucoepidermoid carcinoma. Cancer science 57 23035786
2008 CRTC1/MAML2 fusion transcript in Warthin's tumor and mucoepidermoid carcinoma: evidence for a common genetic association. Genes, chromosomes & cancer 53 18181164
2009 Mucoepidermoid carcinoma of the cervix: another tumor with the t(11;19)-associated CRTC1-MAML2 gene fusion. The American journal of surgical pathology 49 19092631
2018 CRTC1-MAML2 fusion-induced lncRNA LINC00473 expression maintains the growth and survival of human mucoepidermoid carcinoma cells. Oncogene 46 29353885
2013 HER2 and EGFR gene copy number alterations are predominant in high-grade salivary mucoepidermoid carcinoma irrespective of MAML2 fusion status. Histopathology 42 23855785
2022 Both YAP1-MAML2 and constitutively active YAP1 drive the formation of tumors that resemble NF2 mutant meningiomas in mice. Genes & development 41 36008139
2018 CRTC1-MAML2 fusion in mucoepidermoid carcinoma of the breast. Histopathology 41 30380176
2007 Identification of a novel fusion gene MLL-MAML2 in secondary acute myelogenous leukemia and myelodysplastic syndrome with inv(11)(q21q23). Genes, chromosomes & cancer 41 17551948
2016 Reevaluation of MAML2 fusion-negative mucoepidermoid carcinoma: a subgroup being actually hyalinizing clear cell carcinoma of the salivary gland with EWSR1 translocation. Human pathology 37 27769871
2013 CRTC1-MAML2 and CRTC3-MAML2 fusions were not detected in metaplastic Warthin tumor and metaplastic pleomorphic adenoma of salivary glands. The American journal of surgical pathology 37 24121173
2009 Gefitinib as targeted therapy for mucoepidermoid carcinoma of the lung: possible significance of CRTC1-MAML2 oncogene. Lung cancer (Amsterdam, Netherlands) 37 19185385
2020 Pan-Cancer Landscape Analysis Reveals Recurrent KMT2A-MAML2 Gene Fusion in Aggressive Histologic Subtypes of Thymoma. JCO precision oncology 36 32923872
2017 A novel fusion gene CRTC3-MAML2 in hidradenoma: histopathological significance. Human pathology 35 29079171
2022 Mucoepidermoid carcinoma may be devoid of squamoid cells by immunohistochemistry: expanding the histologic and immunohistochemical spectrum of MAML2- rearranged salivary gland tumours. Histopathology 33 36208053
2014 CRTC1/MAML2 gain-of-function interactions with MYC create a gene signature predictive of cancers with CREB-MYC involvement. Proceedings of the National Academy of Sciences of the United States of America 32 25071166
2021 Mucoepidermoid carcinoma of the salivary glands revisited with special reference to histologic grading and CRTC1/3-MAML2 genotyping. Virchows Archiv : an international journal of pathology 30 34231055
2013 Analysis of MAML2 rearrangement in mucoepidermoid carcinoma of the thymus. Human pathology 30 24134933
2021 Salivary mucoepidermoid carcinoma: histological variants, grading systems, CRTC1/3-MAML2 fusions, and clinicopathological features. Histopathology 29 34657306
2010 CRTC1/MAML2 fusion transcript in central mucoepidermoid carcinoma of mandible--diagnostic and histogenetic implications. Annals of diagnostic pathology 28 21074686
2022 Recurrent YAP1::MAML2 fusions in "nodular necrotizing" variants of myxoinflammatory fibroblastic sarcoma: a comprehensive study of 7 cases. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 27 35546636
2016 Cutaneous hidradenoma: a study of 21 neoplasms revealing neither correlation between the cellular composition and CRTC1-MAML2 fusions nor presence of CRTC3-MAML2 fusions. Annals of diagnostic pathology 27 27402217
2021 YAP1-MAML2 Fusion as a Diagnostic Biomarker for Metaplastic Thymoma. Frontiers in oncology 26 34354947
2021 LINC01152 upregulates MAML2 expression to modulate the progression of glioblastoma multiforme via Notch signaling pathway. Cell death & disease 25 33483471
2015 A clear cell variant of mucoepidermoid carcinoma harboring CRTC1-MAML2 fusion gene found in buccal mucosa: report of a case showing a large clear cell component and lacking typical epidermoid cells and intermediate cells. Medical molecular morphology 24 26297211
2014 MAML2 rearrangement in primary pulmonary mucoepidermoid carcinoma and the correlation with FLT1 expression. PloS one 24 24714697
2022 CircRNA_Maml2 promotes the proliferation and migration of intestinal epithelial cells after severe burns by regulating the miR-93-3p/FZD7/Wnt/β-catenin pathway. Burns & trauma 23 35265724
2014 Mucoepidermoid carcinoma does not harbor transcriptionally active high risk human papillomavirus even in the absence of the MAML2 translocation. Head and neck pathology 23 24706055
2015 Functional Variants in Notch Pathway Genes NCOR2, NCSTN, and MAML2 Predict Survival of Patients with Cutaneous Melanoma. Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology 22 25953768
2022 MAML2 Gene Rearrangement Occurs in Nearly All Hidradenomas: A Reappraisal in a Series of 20 Cases. The American Journal of dermatopathology 21 35925563
2012 CRTC1-MAML2 gene fusion in mucoepidermoid carcinoma of the lacrimal gland. Oncology reports 21 22323114
1987 Immunohistochemical demonstration of MAM-3 and MAM-6 antigens in normal human skin appendages and their tumors. Archives of dermatological research 21 3435182
2010 Central mucoepidermoid carcinoma, a case report with molecular analysis of the TORC1/MAML2 gene fusion. Head and neck pathology 20 20625861
2024 Phase separation of YAP-MAML2 differentially regulates the transcriptome. Proceedings of the National Academy of Sciences of the United States of America 19 38315854
2020 YAP1-MAML2-Rearranged Poroid Squamous Cell Carcinoma (Squamoid Porocarcinoma) Presenting as a Primary Parotid Gland Tumor. Head and neck pathology 19 32504288
2023 Malignant undifferentiated epithelioid neoplasms with MAML2 rearrangements: A clinicopathologic study of seven cases demonstrating a heterogenous entity. Genes, chromosomes & cancer 18 36344258
2011 Oncocytic mucoepidermoid carcinoma of the parotid gland with CRTC1-MAML2 fusion transcript: report of a case with review of literature. Human pathology 18 21676434
2022 Diagnostic Value of MAML2 Rearrangements in Mucoepidermoid Carcinoma. International journal of molecular sciences 17 35457138
2021 CRTC1/MAML2 directs a PGC-1α-IGF-1 circuit that confers vulnerability to PPARγ inhibition. Cell reports 17 33626346
2020 Clinicopathological significance of EGFR pathway gene mutations and CRTC1/3-MAML2 fusions in salivary gland mucoepidermoid carcinoma. Histopathology 17 32129900
2020 Ependymoma with C11orf95-MAML2 fusion: presenting with granular cell and ganglion cell features. Brain tumor pathology 17 33221956
2017 First case of B ALL with KMT2A-MAML2 rearrangement: a case report. BMC cancer 16 28535805
2017 Spindle Cell Mucoepidermoid Carcinoma of the Palatine Tonsil With CRTC1-MAML2 Fusion Transcript: Report of a Rare Case in a 17-Year-Old Boy and a Review of the Literature. International journal of surgical pathology 16 28637362
2015 Fluorescence in-situ hybridization identifies Mastermind-like 2 (MAML2) rearrangement in odontogenic cysts with mucous prosoplasia: a pilot study. Histopathology 16 25123064
2017 Assessment of biologically aggressive, recurrent glandular odontogenic cysts for mastermind-like 2 (MAML2) rearrangements: histopathologic and fluorescent in situ hybridization (FISH) findings in 11 cases. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 15 29121421
2017 Central mucoepidermoid carcinoma arising from glandular odontogenic cyst confirmed by analysis of MAML2 rearrangement: A case report. Pathology international 15 29131467
2012 MAML2 rearrangement in Warthin's tumour: a fluorescent in situ hybridisation study of metaplastic variants. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 15 22582766
2020 Clear cell papillary neoplasm of the breast with MAML2 gene rearrangement: Clear cell hidradenoma or low-grade mucoepidermoid carcinoma? Pathology, research and practice 14 32853960
2019 Primary mucoepidermoid carcinoma of the liver with CRTC1-MAML2 fusion: a case report. Diagnostic pathology 14 31351495
1985 Expression of MAM-3 and MAM-6 antigens in endometrial and endocervical adenocarcinomas. Virchows Archiv. A, Pathological anatomy and histopathology 14 3925626
2018 The reliability of MAML2 gene rearrangement in discriminating between histologically similar glandular odontogenic cysts and intraosseous mucoepidermoid carcinomas. Oral surgery, oral medicine, oral pathology and oral radiology 13 30692056
2014 Translocation t(2;11) in CLL cells results in CXCR4/MAML2 fusion oncogene. Blood 13 24855209
2023 Sinonasal Adenosquamous Carcinoma - Morphology and Genetic Drivers Including Low- and High-Risk Human Papillomavirus mRNA, DEK::AFF2 Fusion, and MAML2 Rearrangement. Head and neck pathology 12 36849671
2021 Reevaluation of Salivary Lymphadenoma: A Subgroup Identified as Warthin-like Mucoepidermoid Carcinoma Following Molecular Investigation for MAML2 Rearrangement. Archives of pathology & laboratory medicine 12 32960941
2009 A link between the expression of the stem cell marker HMGA2, grading, and the fusion CRTC1-MAML2 in mucoepidermoid carcinoma. Genes, chromosomes & cancer 12 19521953
2018 "Pancreatic Mucoepidermoid Carcinoma" Is not a Pancreatic Counterpart of CRTC1/3-MAML2 Fusion Gene-related Mucoepidermoid Carcinoma of the Salivary Gland, and May More Appropriately be Termed Pancreatic Adenosquamous Carcinoma With Mucoepidermoid Carcinoma-like Features. The American journal of surgical pathology 11 30138216
2015 Molecular Basis for the Mechanism of Constitutive CBP/p300 Coactivator Recruitment by CRTC1-MAML2 and Its Implications in cAMP Signaling. Biochemistry 11 26274502
2021 Adenocarcinoma of the minor salivary gland with concurrent MAML2 and EWSR1 alterations. Journal of pathology and translational medicine 10 33472334
2021 Whole Exome Sequencing Identifies Somatic Variants in an Oral Composite Hemangioendothelioma Characterized by YAP1-MAML2 Fusion. Head and neck pathology 10 34791601
2019 High Grade Transformation in Mucoepidermoid Carcinoma of the Minor Salivary Gland with Polyploidy of the Rearranged MAML2 Gene. Head and neck pathology 10 31535311
2009 Chromosomal imbalances, 11q21 rearrangement and MECT1-MAML2 fusion transcript in mucoepidermoid carcinomas of the salivary gland. Oncology reports 10 19578770
2023 Loss of YAP1 C-terminus expression as an ancillary marker for metaplastic thymoma: a potential pitfall in detecting YAP1::MAML2 gene rearrangement. Histopathology 9 37565303
2022 Establishment of a patient-derived mucoepidermoid carcinoma cell line with the CRTC1-MAML2 fusion gene. Molecular and clinical oncology 9 35251626
2022 Diagnostic Reliability of CRTC1/3::MAML2 Gene Fusion Transcripts in Discriminating Histologically Similar Intraosseous Mucoepidermoid Carcinoma from Glandular Odontogenic Cyst: A Systematic Review and Meta-analysis. Head and neck pathology 9 36357765
2017 Mucoepidermoid carcinoma of the sublingual gland harboring a translocation of the MAML2 gene: A case report. Oncology letters 9 28927048
2017 Sclerosing Mucoepidermoid Carcinoma in the Parotid Gland With CRTC1-MAML2 Fusion: A Case Report. International journal of surgical pathology 9 29169286
1989 Immunohistochemical expression of MAM-3 and MAM-6 antigens in salivary gland tumours. Virchows Archiv. A, Pathological anatomy and histopathology 9 2508309
2023 Atypical Intraparenchymal Meningioma with YAP1-MAML2 Fusion in a Young Adult Male: A Case Report and Mini Literature Review. International journal of molecular sciences 8 37628996
2024 MAML2 gene rearrangement occurs in all Warthin-like mucoepidermoid carcinoma: A reappraisal in a series of 29 cases. Heliyon 7 38304779
2022 YAP1-MAML2 fusion in a pediatric NF2-wildtype intraparenchymal brainstem schwannoma. Acta neuropathologica communications 7 35986430
2023 Pathogenic variations in MAML2 and MAMLD1 contribute to congenital hypothyroidism due to dyshormonogenesis by regulating the Notch signalling pathway. Journal of medical genetics 6 36898841
2022 Pediatric meningioma with a Novel MAML2-YAP1 fusion variant: a case report and literature review. BMC pediatrics 6 36463108
1991 Immunohistochemical localization of MAM-3 and MAM-6 antigens in adenoid cystic carcinoma. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 6 1849991
1991 Immunohistochemical distribution of MAM-3 and MAM-6 antigens in developing salivary glands of the human fetus. The Histochemical journal 6 1917564
2023 Salivary Gland Neoplasms With a Unique Trabecular Histology and MAML2 Translocation : A Trabecular Variant of a Mucoepidermoid Carcinoma. The American journal of surgical pathology 5 37589282
2021 The Multifaceted Appearance of Supratentorial Ependymoma with ZFTA-MAML2 Fusion. Free neuropathology 5 37284641
2020 Inv(11)(q21q23); KMT2A-MAML2, a Recurrent Genetic Abnormality in T-Cell Therapy-related Acute Lymphoblastic Leukemia. Journal of pediatric hematology/oncology 5 31343482
2023 YAP1::MAML2 fusions in poromatosis: A report of two patients. Journal of cutaneous pathology 4 36723803
2023 Significance of YAP1-MAML2 rearrangement and GTF2I mutation in the diagnosis and differential diagnosis of metaplastic thymoma. Annals of medicine 4 37489594
2020 Ameloblastoma with mucous cells: A clinicopathological, BRAF mutation, and MAML2 rearrangement study. Oral diseases 4 31954088

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