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Rabbit Anti-FIP1L1 antibody
The Component of the Cleavage and Polyadenylation Specificity Factor (CPSF) complex plays an important role in the 3'-end formation of pre-mRNA. This complex recognizes the AAUAAA signal sequence and interacts with poly(A) polymerase to process and add to the poly(A) tail. FIP1L1 (FIP1-like 1), also known as Pre-mRNA 3'-end-processing factor FIP1, FIP1 (Factor interacting with PAP) and RHE (Rearranged in hypereosinophilia), is a 594 amino acid nuclear protein that is a component of the CPSF complex. Within the complex, FIP1L1 contributes to the poly(A) recognition and stimulates poly(A) addition. Fusion of the genes encoding FIP1L1 and PDGFRA due to an interstitial deletion on chromosome 4q12 is the cause of hypereosinophilia syndrome, a rare blood disorder characterized by continuous overproduction of eosinophils in the bone marrow that leads to tissue infiltration and organ damage. There are three isoforms of FIP1L1 that are produced as a result of alternative splicing events.
Function:
FIP1L1 is an integral subunit of the cleavage and polyadenylation specificity factor (CPSF) complex. CPSF is a multisubunit complex that binds to the canonical AAUAAA hexamer and to U-rich upstream sequence elements on the pre-mRNA and interacts with poly(A) polymerase and other factors to bring about cleavage and poly(A) addition. FIP1L1 binds to U-rich RNA sequence elements surrounding the poly(A) site and contributes to poly(A) site recognition and stimulates poly(A) addition. FIP1L1 may act to tether poly(A) polymerase to the CPSF complex. A fusion of FIP1L1 and PDGFRA (FIP1L1-PDGFRA) is a recurrent molecular lesion in eosinophilia-associated myeloproliferative disorders.
Subunit:
Component of the cleavage and polyadenylation specificity factor (CPSF) complex, composed of CPSF1, CPSF2, CPSF3, CPSF4 and FIP1L1. Found in a complex with CPSF1, FIP1L1 and PAPOLA. Interacts with CPSF1, CPSF4, CSTF2, CSTF3 and PAPOLA.
Subcellular Location:
Nuclear
DISEASE:
Note=A chromosomal aberration involving FIP1L1 is found in some cases of hypereosinophilic syndrome. Interstitial chromosomal deletion del(4)(q12q12) causes the fusion of FIP1L1 and PDGFRA (FIP1L1-PDGFRA).
Similarity:
Belongs to the FIP1 family.
SWISS:
Q6UN15
Gene ID:
81608
Database links:
Entrez Gene: 81608 Human
Omim: 607686 Human
SwissProt: Q6UN15 Human
Unigene: 624245 Human
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