Protein Name factor interacting with PAPOLA and CPSF1
Organism Homo sapiens
Gene ID 81608
Gene Symbol

FIP1L1

UniProt Q6UN15 (FIP1_HUMAN)
Relationships Total Number of functionally related compound(s) : 167
Total Number of Articles : 246
Description

factor interacting with PAPOLA and CPSF1

Gene Summary

This gene encodes a subunit of the CPSF (cleavage and polyadenylation specificity factor) complex that polyadenylates the 3' end of mRNA precursors. This gene, the homolog of yeast Fip1 (factor interacting with PAP), binds to U-rich sequences of pre-mRNA and stimulates poly(A) polymerase activity. Its N-terminus contains a PAP-binding site and its C-terminus an RNA-binding domain. An interstitial chromosomal deletion on 4q12 creates an in-frame fusion of human genes FIP1L1 and PDGFRA (platelet-derived growth factor receptor, alpha). The FIP1L1-PDGFRA fusion gene encodes a constitutively activated tyrosine kinase that joins the first 233 amino acids of FIP1L1 to the last 523 amino acids of PDGFRA. This gene fusion and chromosomal deletion is the cause of some forms of idiopathic hypereosinophilic syndrome (HES). This syndrome, recently reclassified as chronic eosinophilic leukemia (CEL), is responsive to treatment with tyrosine kinase inhibitors. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Oct 2008]

synonyms
  • pre-mRNA 3'-end-processing factor FIP1
  • FIP1 like 1
  • FIP1-like 1 protein
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Properties