Molecular Profile Detail

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Profile Name KMT2A - MLLT3 SRSF2 P95H
Gene Variant Detail

KMT2A - MLLT3 (gain of function)

SRSF2 P95H (loss of function)

Relevant Treatment Approaches

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
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
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 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 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 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
Clinical Trial Phase Therapies Title Recruitment Status