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Gene Review

FIP1L1  -  factor interacting with PAPOLA and CPSF1

Homo sapiens

Synonyms: DKFZp586K0717, FIP1, FIP1-like 1 protein, Factor interacting with PAP, Pre-mRNA 3'-end-processing factor FIP1, ...
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Disease relevance of FIP1L1


High impact information on FIP1L1


Chemical compound and disease context of FIP1L1


Biological context of FIP1L1


Anatomical context of FIP1L1


Associations of FIP1L1 with chemical compounds


Other interactions of FIP1L1

  • Screening for the FIP1L1-PDGFRA rearrangement and Asp816Val mutation will advance rational therapy decisions in SMCD [1].
  • These observations suggest that the FIP1L1-PDGFRA rearrangement occurs in an early hematopoietic progenitor and suggests that the molecular pathogenesis for a subset of SMCD patients is similar to that of HES [1].
  • Retrospectively, a CHIC2 deletion and the FIP1L1/PDGFRalpha fusion gene-product were demonstrable by FISH analysis and RT-PCR, respectively [18].
  • Imatinib is an excellent candidate for first line treatment of Loeffler's endocarditis, especially when the FIP1L1/PDGFA fusion gene is detected [13].
  • This case provides further evidence for the potential of co-existence of SM with a primary eosinophilic disorder (CEL) defined by the FIP1L1/PDGFRalpha fusion gene [18].

Analytical, diagnostic and therapeutic context of FIP1L1


  1. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy. Pardanani, A., Ketterling, R.P., Brockman, S.R., Flynn, H.C., Paternoster, S.F., Shearer, B.M., Reeder, T.L., Li, C.Y., Cross, N.C., Cools, J., Gilliland, D.G., Dewald, G.W., Tefferi, A. Blood (2003) [Pubmed]
  2. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. Cools, J., DeAngelo, D.J., Gotlib, J., Stover, E.H., Legare, R.D., Cortes, J., Kutok, J., Clark, J., Galinsky, I., Griffin, J.D., Cross, N.C., Tefferi, A., Malone, J., Alam, R., Schrier, S.L., Schmid, J., Rose, M., Vandenberghe, P., Verhoef, G., Boogaerts, M., Wlodarska, I., Kantarjian, H., Marynen, P., Coutre, S.E., Stone, R., Gilliland, D.G. N. Engl. J. Med. (2003) [Pubmed]
  3. FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Pardanani, A., Brockman, S.R., Paternoster, S.F., Flynn, H.C., Ketterling, R.P., Lasho, T.L., Ho, C.L., Li, C.Y., Dewald, G.W., Tefferi, A. Blood (2004) [Pubmed]
  4. Myeloid blast crisis evolving during imatinib treatment of an FIP1L1-PDGFR alpha-positive chronic myeloproliferative disease with prominent eosinophilia. von Bubnoff, N., Sandherr, M., Schlimok, G., Andreesen, R., Peschel, C., Duyster, J. Leukemia (2005) [Pubmed]
  5. Platelet-derived growth factor receptor-alpha-associated hypereosinophilic syndrome and lymphomatoid papulosis. McPherson, T., Cowen, E.W., McBurney, E., Klion, A.D. Br. J. Dermatol. (2006) [Pubmed]
  6. Analysis of a noncanonical poly(A) site reveals a tripartite mechanism for vertebrate poly(A) site recognition. Venkataraman, K., Brown, K.M., Gilmartin, G.M. Genes Dev. (2005) [Pubmed]
  7. Discovery of a fusion kinase in EOL-1 cells and idiopathic hypereosinophilic syndrome. Griffin, J.H., Leung, J., Bruner, R.J., Caligiuri, M.A., Briesewitz, R. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  8. The FIP1L1-PDGFRA fusion gene cooperates with IL-5 to induce murine hypereosinophilic syndrome (HES)/chronic eosinophilic leukemia (CEL)-like disease. Yamada, Y., Rothenberg, M.E., Lee, A.W., Akei, H.S., Brandt, E.B., Williams, D.A., Cancelas, J.A. Blood (2006) [Pubmed]
  9. Sorafenib is a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Lierman, E., Folens, C., Stover, E.H., Mentens, N., Van Miegroet, H., Scheers, W., Boogaerts, M., Vandenberghe, P., Marynen, P., Cools, J. Blood (2006) [Pubmed]
  10. Multilineage involvement of the fusion gene in patients with FIP1L1/PDGFRA-positive hypereosinophilic syndrome. Robyn, J., Lemery, S., McCoy, J.P., Kubofcik, J., Kim, Y.J., Pack, S., Nutman, T.B., Dunbar, C., Klion, A.D. Br. J. Haematol. (2006) [Pubmed]
  11. The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management. Gotlib, J., Cools, J., Malone, J.M., Schrier, S.L., Gilliland, D.G., Coutré, S.E. Blood (2004) [Pubmed]
  12. Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial. Droogendijk, H.J., Kluin-Nelemans, H.J., van Doormaal, J.J., Oranje, A.P., van de Loosdrecht, A.A., van Daele, P.L. Cancer (2006) [Pubmed]
  13. Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. Rotoli, B., Catalano, L., Galderisi, M., Luciano, L., Pollio, G., Guerriero, A., D'Errico, A., Mecucci, C., La Starza, R., Frigeri, F., Di Francia, R., Pinto, A. Leuk. Lymphoma (2004) [Pubmed]
  14. Chronic eosinophilic leukemia with the FIP1L1-PDGFRalpha fusion gene in a patient with a history of combination chemotherapy. Tanaka, Y., Kurata, M., Togami, K., Fujita, H., Watanabe, N., Matsushita, A., Maeda, A., Nagai, K., Sada, A., Matsui, T., Takahashi, T. Int. J. Hematol. (2006) [Pubmed]
  15. The EOL-1 cell line as an in vitro model for the study of FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. Cools, J., Quentmeier, H., Huntly, B.J., Marynen, P., Griffin, J.D., Drexler, H.G., Gilliland, D.G. Blood (2004) [Pubmed]
  16. Rhe irreplaceable image: Superficial thrombosis of a varicose vein of the abdominal wall as the first sign of an otherwise occult locally-advanced ovarian cancer. Porta, C., Barone, M., Bressan, M.A. Haematologica (2001) [Pubmed]
  17. Activity of AMN107, a novel aminopyrimidine tyrosine kinase inhibitor, against human FIP1L1-PDGFR-alpha-expressing cells. Verstovsek, S., Giles, F.J., Quint??s-Cardama, A., Manshouri, T., Huynh, L., Manley, P., Cortes, J., Tefferi, A., Kantarjian, H. Leuk. Res. (2006) [Pubmed]
  18. Systemic mastocytosis (SM) associated with chronic eosinophilic leukemia (SM-CEL): detection of FIP1L1/PDGFRalpha, classification by WHO criteria, and response to therapy with imatinib. Florian, S., Esterbauer, H., Binder, T., Müllauer, L., Haas, O.A., Sperr, W.R., Sillaber, C., Valent, P. Leuk. Res. (2006) [Pubmed]
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