Signaling by FLT3 fusion proteins

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R-HSA-9703465
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Homo sapiens
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In addition to internal tandem duplications and activating point mutations, FLT3 is also subject at low frequency to translocations that generate fusion proteins. These fusion proteins occur in some chronic myeloid leukemias as well as myeloid neoplasms with eosinophilia, and generate constitutively active proteins by virtue of fusing a N-terminal partner encoding a dimerization domain with the intracellular region of FLT3 (reviewed in Reiter and Gotlib, 2017; Kazi and Roonstrand, 2019). To date, 6 fusion partner genes of FLT3 have been identified: ETV6, TRIP11, MYO18A, SPTBN1, GOLGB1 and ZMYM2 (Balwin et al, 2007; Vu et al, 2006; Walz et al, 2011; Falchi et al, 2014; Chung et al, 2017; Troadec et al, 2017; Grand et al, 2007; Jawhar et al, 2017; Zhang et al, 2018).

Literature References
PubMed ID Title Journal Year
16761019 FLT3 is fused to ETV6 in a myeloproliferative disorder with hypereosinophilia and a t(12;13)(p13;q12) translocation

Sato, Y, Vu, HA, Motoji, T, Sakaki, Y, Xinh, PT, Tokunaga, K, Toyoda, A, Masuda, M

2006
24854988 ETV6-FLT3 fusion gene-positive, eosinophilia-associated myeloproliferative neoplasm successfully treated with sorafenib and allogeneic stem cell transplant

Popat, U, Falchi, L, Patel, KP, Lyle, LM, Luthra, R, Mehrotra, M, Newberry, KJ, Verstovsek, S, Lu, G

2014
21705501 Response of ETV6-FLT3-positive myeloid/lymphoid neoplasm with eosinophilia to inhibitors of FMS-like tyrosine kinase 3

Score, J, Erben, P, Haferlach, C, Gesk, S, Ritter, M, Telford, N, Haferlach, T, Reiter, A, Cross, NC, Metzgeroth, G, Hofmann, WK, Walz, C, Bloor, A, Hochhaus, A

2011
31066629 FMS-like Tyrosine Kinase 3/FLT3: From Basic Science to Clinical Implications

Kazi, JU, Rönnstrand, L

Physiol. Rev. 2019
27795560 A novel t(3;13)(q13;q12) translocation fusing FLT3 with GOLGB1: toward myeloid/lymphoid neoplasms with eosinophilia and rearrangement of FLT3?

Petit, B, Dobbelstein, S, Chauzeix, J, Touati, M, Gachard, N, Faumont, N, Bertrand, P, Troadec, E, Trimoreau, F, Bordessoule, D, Bastard, C, Feuillard, J

2017
17268528 Transgenic mice expressing Tel-FLT3, a constitutively activated form of FLT3, develop myeloproliferative disease

Collector, M, Racke, F, Small, S, Sharkis, SJ, Whartenby, KA, Li, L, Baldwin, BR, Tse, KF, Huso, D, Small, D

2007
17764812 A constitutively active SPTBN1-FLT3 fusion in atypical chronic myeloid leukemia is sensitive to tyrosine kinase inhibitors and immunotherapy

Grand, FH, Zhang, L, Russell, NH, Cross, NC, Chase, A, Iqbal, S

2007
30559310 Two myeloid leukemia cases with rare FLT3 fusions

Olson, S, Zhang, H, Moore, S, Long, N, Paliga, A, Hobbs, E, Tyner, JW, Dao, KT

2018
28751768 Cytogenetically cryptic ZMYM2-FLT3 and DIAPH1-PDGFRB gene fusions in myeloid neoplasms with eosinophilia

Jawhar, M, Tapper, W, Metzgeroth, G, Chacko, J, Score, J, Chase, A, Kreuzer, KA, Knut, M, Naumann, N, Cross, NCP, Schneider, B, Hofmann, WK, Fabarius, A, Hallek, M, Drexler, HG, Ghazzawi, M, Wallis, L, Reiter, A

2017
29025582 A novel TRIP11-FLT3 fusion in a patient with a myeloid/lymphoid neoplasm with eosinophilia

Fisk, DG, Fire, AZ, Gojenola, L, Li, X, Arber, DA, Gotlib, J, Roskin, KM, Von Gehr, A, Hou, Y, Zehnder, JL, Bangs, CD, Cherry, AM, Ohgami, RS, Merker, JD, Chung, A

2017
28028030 Myeloid neoplasms with eosinophilia

Gotlib, J, Reiter, A

2017
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cancer DOID:162 malignant tumor, malignant neoplasm, primary cancer
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