Overview:

FIP1L1 or factor interacting with PAPOLA and CPSF1 is a subunit of the CPSF (cleavage and polyadenylation specificity factor) complex that polyadenylates the 3' end of mRNA precursors. The FIP1L1-PDGFRA fusion gene encodes a constitutively activated tyrosine kinase that transforms hematopoietic cells and is inhibited by imatinib in which fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome (1). FIP1L1-PDGFR-alpha requires disruption of the juxtamembrane protein of PDGFR-alpha and is FIP1L1-independent (2).

Gene Aliases:

FIP1L1: FIP1, RHE, DKFZp586K0717
PDGFRA: CD140A, PDGFR2, MGC74795, Rhe-PDGFRA

Genbank Number:

AM392758
NM_006206

References:

1. Cools, J. et.al: A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. New Eng. J. Med. 348: 1201-1214, 2003.

2. Stover, E. H. et.al: FIP1L1-PDGFR-alpha requires disruption of the juxtamembrane protein of PDGFR-alpha and is FIP1L1-independent. Proc. Nat. Acad. Sci. 103: 8078-8083, 2006.