Autophosphorylation of cytosolic PDGFRA and PDGFRB fusion proteins

Stable Identifier
Reaction [transition]
Homo sapiens
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Where examined, cytosolic fusion proteins of PDGFRA and B have been shown to undergo constitutive trans-autophosphorylation in the absence of ligand (Cools et al, 2003; Stover et al, 2006; Salemi et al, 2009; reviewed in Reilly et al, 2003; Wang et al, 2016; Appiah-Kubi et al, 2017). Although downstream signaling has not been investigated in detail, the FIP1L1-PDGFRA fusion has been shown to signal through STAT5 and its expression in Ba/F3 murine cell line promotes oncogenic transformation (Cools et al, 2003).
Literature References
PubMed ID Title Journal Year
27170215 The platelet-derived growth factors (PDGFs) and their receptors (PDGFRs) are major players in oncogenesis, drug resistance, and attractive oncologic targets in cancer

Yao, X, Chen, Y, Wu, M, Qian, H, Wang, Y, Wu, Y, Appiah-Kubi, K

Growth Factors 2016
16690743 Activation of FIP1L1-PDGFRalpha requires disruption of the juxtamembrane domain of PDGFRalpha and is FIP1L1-independent

Marynen, P, Folens, C, Gilliland, DG, Williams, IR, Mentens, N, Cools, J, Stover, EH, Lee, BH, Chen, J

Proc. Natl. Acad. Sci. U.S.A. 2006
12660384 A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome

Marynen, P, Boogaerts, M, Griffin, JD, Galinsky, I, Cortes, J, Coutré, SE, Kantarjian, H, Schmid, J, Malone, J, Schrier, SL, Cools, J, Stover, EH, Wlodarska, I, Kutok, J, Rose, M, Verhoef, G, Vandenberghe, P, Gotlib, J, Gilliland, DG, Alam, R, Clark, J, Legare, RD, Cross, NC, Stone, R, Deangelo, DJ, Tefferi, A

N. Engl. J. Med. 2003
19210352 A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome

Scapozza, L, Simon, D, Salemi, S, Moretti, L, Simon, HU, Yousefi, S, Schmid, I

Allergy 2009
28010895 Platelet-derived growth factor receptors (PDGFRs) fusion genes involvement in hematological malignancies

Yao, X, Chen, Y, Wu, M, Qian, H, Wang, Y, Wu, Y, Appiah-Kubi, K, Lan, T

Crit. Rev. Oncol. Hematol. 2017
14556779 Receptor tyrosine kinases in normal and malignant haematopoiesis

Reilly, JT

Blood Rev. 2003
Catalyst Activity

protein tyrosine kinase activity of cytosolic PDGFR fusion dimers [cytosol]

Functional status

Gain of function of cytosolic PDGFR fusion dimers [cytosol]

Disease Entity
Name Identifier Synonyms
cancer DOID:162 malignant tumor, malignant neoplasm, primary cancer
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