Signaling by FLT3 fusion proteins

Stable Identifier
R-HSA-9703465
Type
Pathway
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this pathway in the Pathway Browser

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

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

2006
28751768 Cytogenetically cryptic ZMYM2-FLT3 and DIAPH1-PDGFRB gene fusions in myeloid neoplasms with eosinophilia

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

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

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

2017
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?

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

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

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

2007
21705501 Response of ETV6-FLT3-positive myeloid/lymphoid neoplasm with eosinophilia to inhibitors of FMS-like tyrosine kinase 3

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

2011
30559310 Two myeloid leukemia cases with rare FLT3 fusions

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

2018
17764812 A constitutively active SPTBN1-FLT3 fusion in atypical chronic myeloid leukemia is sensitive to tyrosine kinase inhibitors and immunotherapy

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

2007
24854988 ETV6-FLT3 fusion gene-positive, eosinophilia-associated myeloproliferative neoplasm successfully treated with sorafenib and allogeneic stem cell transplant

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

2014
28028030 Myeloid neoplasms with eosinophilia

Reiter, A, Gotlib, J

2017
Participants
Participant Of
Disease
Name Identifier Synonyms
cancer 162 malignant tumor, malignant neoplasm, primary cancer
Authored
Reviewed
Created
Cite Us!