Gene Variant Detail

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Gene KMT2A MLLT3
Variant KMT2A - MLLT3
Impact List fusion
Protein Effect gain of function
Gene Variant Descriptions KMT2A-MLLT3 (also referred to as MLL-AF9) results from the fusion of KMT2A and MLLT3, resulting in a gain of function protein that induces cell proliferation and drives tumorigenesis in culture (PMID: 11607819).
Associated Drug Resistance

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No Variant Reference Transcript is Available.
No transcript is Available.

Filtering

  • Case insensitive filtering will display rows where any text in any cell matches the filter term
  • Simple literal full or partial string matches
  • Separate multiple filter terms with a spaces, order doesn't matter (a b c and c b a are equivalent )
  • Filtering will only apply to rows that are already loaded on the page, filtering has no impact on query parameters
  • Use quotes to match a longer phrase which contains spaces "mtor c1483f"

Sorting

  • Generally, the default sort order for tables is set to be first column ascending, however, specific tables may set a different default sort order.
  • Click on any column header arrows to sort by that column
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Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
KMT2A - MLLT3 acute myeloid leukemia sensitive MI-503 Preclinical - Patient cell culture Actionable In a preclinical study, MI-503 inhibited growth of an acute myeloid leukemia (AML) cell line and primary AML samples harboring KMT2A-MLLT3 (MLL-AF9) in culture (PMID: 25817203). 25817203
KMT2A - MLLT3 acute myeloid leukemia sensitive I-BET151 + SGC0946 Preclinical - Patient cell culture Actionable In a preclinical study, I-BET151 and SGC0946 synergistically inhibited proliferation of patient-derived acute myeloid leukemia cells harboring KMT2A-MLLT3 in culture, and prolonged survival in animal models of mouse KMT2A-MLLT3-positive leukemia (PMID: 27294782). 27294782
KMT2A - MLLT3 leukemia sensitive IRAK1/4 Preclinical Actionable In a preclinical study, a transgenic leukemic mouse model harboring KMT2A-MLLT3 demonstrated sensitivity to IRAK1/4, resulting in delayed progression and improved survival (PMID: 28065413). 28065413
KMT2A - MLLT3 leukemia sensitive MI-503 Preclinical Actionable In a preclinical study, MI-503 reduced progression and improved survival of a mouse leukemia model expressing KMT2A-MLLT3 (MLL-AF9) (PMID: 25817203). 25817203
KMT2A - MLLT3 acute myeloid leukemia not applicable N/A Guideline Prognostic KMT2A-MLLT3 fusions are associated with an intermediate prognosis in patients with non-APL acute myeloid leukemia (NCCN.org). detail...
KMT2A - MLLT3 acute myeloid leukemia sensitive E7107 + GSK3203591 Preclinical - Cell culture Actionable In a preclinical study, E7107 and GSK3203591 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia cells in culture, regardless of SRSF2 mutation status (PMID: 31408619). 31408619
KMT2A - MLLT3 acute myeloid leukemia sensitive GSK3203591 + MS023 Preclinical - Cell culture Actionable In a preclinical study, MS023 and GSK3203591 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia (AML) cells and human AML cell lines harboring KMT2A-MLLT3 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 acute myeloid leukemia sensitive Dinaciclib Preclinical Actionable In a preclinical study, treatment with Dinaciclib resulted in induced cell death of acute myeloid leukemia cells harboring KMT2A-MLLT3 in culture (PMID: 26627013). 26627013
KMT2A - MLLT3 acute myeloid leukemia sensitive E7107 + MS023 Preclinical - Cell culture Actionable In a preclinical study, MS023 and E7107 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia cells in culture, regardless of SRSF2 mutation status (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive GSK3203591 Preclinical - Cell culture Actionable In a preclinical study, murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells expressing SRSF2 P95H demonstrated increased sensitivity to GSK3203591 compared to cells expressing wild-type SRSF2 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive E7107 Preclinical Actionable In a preclinical study, E7107 treatment decreased tumor burden and prolonged survival in an isogenic mouse model of acute myeloid leukemia expressing KMT2A-MLLT3 (reported as MLL-AF9) and SRSF2 P95H, but not in a model expressing KMT2A-MLLT3 and wild-type SRSF2 (PMID: 27135740). 27135740
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive E7107 Preclinical - Cell culture Actionable In a preclinical study, murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells expressing SRSF2 P95H demonstrated increased sensitivity to E7107 compared to cells expressing wild-type SRSF2 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive H3B-8800 Preclinical Actionable In a preclinical study, H3B-8800 inhibited tumor growth in transplant animal models of mouse acute myeloid leukemia cells harboring SRSF2 P95H and KMT2A-MLLT3 (MLL-AF9) while had no effect in SRSF2 wild-type controls (Blood 2016 128:966). detail...
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive EPZ015666 Preclinical Actionable In a preclinical study, EPZ015666 (GSK3235025) treatment decreased SDMA levels and prolonged survival in a mouse model of KMT2A-MLLT3 (MLL-AF9)-driven acute myeloid leukemia harboring SRSF2 P95H, but not in a SRSR2 wild-type model (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive MS023 Preclinical Actionable In a preclinical study, MS023 inhibited survival of murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells harboring SRSF2 P95H in culture and delayed disease progression in mouse models, but not in wild-type SRSF2 models (PMID: 31408619). 31408619
Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
KMT2A fusion acute myeloid leukemia sensitive CDKI-73 Preclinical - Cell line xenograft Actionable In a preclinical study, CDKI-73 treatment inhibited Cdk9 kinase activity and viability, and induced apoptosis in acute myeloid leukemia cell lines harboring KMT2A fusions in culture, and inhibited tumor growth and induced regression in a cell line xenograft model (PMID: 30194564). 30194564
KMT2A fusion acute myeloid leukemia sensitive I-BET151 + SGC0946 Preclinical - Patient cell culture Actionable In a preclinical study, I-BET151 and SGC0946 synergistically inhibited proliferation of patient-derived acute myeloid leukemia cells harboring KMT2A fusions in culture (PMID: 27294782). 27294782
KMT2A fusion acute myeloid leukemia predicted - sensitive MI-503 Preclinical - Cell culture Actionable In a preclinical study, MI-503 inhibited growth of several acute myeloid leukemia (AML) cell lines and primary AML samples harboring KMT2A (MLL) fusions in culture (PMID: 25817203). 25817203
KMT2A rearrange acute leukemia predicted - sensitive Pinometostat Case Reports/Case Series Actionable In a Phase I trial, Pinometostat (EPZ-5676) treatment resulted in complete remission in 2 patients with KMT2A rearranged (both with t(11;19)) acute leukemia (PMID: 29724899; NCT01684150). 29724899
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Romidepsin Preclinical - Cell line xenograft Actionable In a preclinical study, Istodax (romidepsin) enhanced the effects of Cytosar-U (cytarabine) in acute lymphocytic leukemia cell lines harboring a KMT2A rearrangement in culture, and in cell line xenograft models, demonstrating decreased leukemic cells by 73% (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia decreased response Romidepsin Preclinical - Cell line xenograft Actionable In a preclinical study, Istodax (romidepsin) resulted in minimal activity in acute lymphocytic leukemia cell lines harboring a KMT2A rearrangement in culture, and in cell line xenograft models, demonstrating decreased leukemic cells by 16% (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine Preclinical - Cell line xenograft Actionable In a preclinical study, Cytosar-U (cytarabine) decreased leukemic cells by 66% in acute lymphocytic leukemia cell line xenograft models harboring a KMT2A rearrangement (PMID: 27443263). 27443263
KMT2A rearrange acute myeloid leukemia predicted - sensitive KO-539 Preclinical - Cell line xenograft Actionable In a preclinical study, KO-539 inhibited growth of KMT2A (MLL1)-rearranged acute myeloid leukemia cells in culture, and prolonged survival in cell-line xenograft models (AACR; Mol Cancer Ther 2018;17(1 Suppl):Abstract nr LB-A27). detail...
KMT2A rearrange leukemia predicted - sensitive I-CBP112 + JQ1 Preclinical Actionable In a preclinical study, I-CBP112 sensitized leukemia cells harboring KMT2A fusions to JQ1, resulting in decreased cell growth in culture (PMID: 26552700). 26552700
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Panobinostat Preclinical - Cell culture Actionable In a preclinical study, Farydak (panobinostat) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cells harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Mocetinostat Preclinical - Cell culture Actionable In a preclinical study, Mocetinostat (MGCD0103) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cell lines harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Dacinostat Preclinical - Cell culture Actionable In a preclinical study, Dacinostat (LAQ824) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cell lines harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange leukemia predicted - sensitive Doxorubicin + I-CBP112 Preclinical Actionable In a preclinical study, I-CBP112 sensitized leukemia cells harboring KMT2A fusions to Doxorubicin, leading to decreased cell growth in culture (PMID: 26552700). 26552700
KMT2A rearrange acute myeloid leukemia not applicable N/A Clinical Study Prognostic In multiple clinical studies, KMT2A rearrangements, specifically partial tandem duplications, were associated with a poor overall survival in acute myeloid leukemia patients (PMID: 24487413, PMID: 22915647, PMID: 22417203, PMID: 21881046). 21881046 24487413 22417203 22915647
KMT2A rearrange acute myeloid leukemia not applicable N/A Guideline Prognostic KMT2A rearrangements (t(v;11q23.3)) are associated with a poor/adverse prognosis in patients with non-APL acute myeloid leukemia (NCCN.org). detail...
Molecular Profile Protein Effect Treatment Approaches
KMT2A - MLLT3 gain of function
KMT2A - MLLT3 SRSF2 P95H