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Protein

Pre-mRNA 3'-end-processing factor FIP1

Gene

FIP1L1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Component of the cleavage and polyadenylation specificity factor (CPSF) complex that plays a key role in pre-mRNA 3'-end formation, recognizing the AAUAAA signal sequence and interacting with poly(A) polymerase and other factors to bring about cleavage and poly(A) addition. FIP1L1 contributes to poly(A) site recognition and stimulates poly(A) addition. Binds to U-rich RNA sequence elements surrounding the poly(A) site. May act to tether poly(A) polymerase to the CPSF complex.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei339 – 340Breakpoint for interstitial deletion to form the FIP1L1-PDGFRA fusion protein2

GO - Molecular functioni

  • poly(A) RNA binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

mRNA processing

Keywords - Ligandi

RNA-binding

Enzyme and pathway databases

ReactomeiR-HSA-109688. Cleavage of Growing Transcript in the Termination Region.
R-HSA-159231. Transport of Mature mRNA Derived from an Intronless Transcript.
R-HSA-72163. mRNA Splicing - Major Pathway.
R-HSA-72187. mRNA 3'-end processing.
R-HSA-77595. Processing of Intronless Pre-mRNAs.
SIGNORiQ6UN15.

Names & Taxonomyi

Protein namesi
Recommended name:
Pre-mRNA 3'-end-processing factor FIP1
Short name:
hFip1
Alternative name(s):
FIP1-like 1 protein
Factor interacting with PAP
Rearranged in hypereosinophilia
Gene namesi
Name:FIP1L1
Synonyms:FIP1, RHE
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:19124. FIP1L1.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Involvement in diseasei

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).

Organism-specific databases

DisGeNETi81608.
MalaCardsiFIP1L1.
MIMi607685. phenotype.
OpenTargetsiENSG00000145216.
Orphaneti520. Acute promyelocytic leukemia.
314950. Primary hypereosinophilic syndrome.
PharmGKBiPA134875694.

Polymorphism and mutation databases

BioMutaiFIP1L1.
DMDMi74749365.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002150371 – 594Pre-mRNA 3'-end-processing factor FIP1Add BLAST594

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei85PhosphoserineBy similarity1
Modified residuei87PhosphoserineBy similarity1
Modified residuei89PhosphoserineBy similarity1
Modified residuei304PhosphoserineBy similarity1
Modified residuei426PhosphotyrosineCombined sources1
Modified residuei492PhosphoserineCombined sources1
Modified residuei494PhosphothreonineCombined sources1
Modified residuei496PhosphoserineCombined sources1
Modified residuei500PhosphoserineCombined sources1
Modified residuei554PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ6UN15.
MaxQBiQ6UN15.
PaxDbiQ6UN15.
PeptideAtlasiQ6UN15.
PRIDEiQ6UN15.

PTM databases

iPTMnetiQ6UN15.
PhosphoSitePlusiQ6UN15.

Miscellaneous databases

PMAP-CutDBQ6UN15.

Expressioni

Gene expression databases

BgeeiENSG00000145216.
CleanExiHS_FIP1L1.
ExpressionAtlasiQ6UN15. baseline and differential.
GenevisibleiQ6UN15. HS.

Organism-specific databases

HPAiHPA037475.
HPA058202.

Interactioni

Subunit structurei

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 and CSTF3 (PubMed:14749727). Interacts with AHCYL1 (when phosphorylated); the interaction is direct and associates AHCYL1 with the CPSF complex and RNA (PubMed:19224921). Interacts with PAPOLA; the interaction seems to be increased by the interaction with AHCYL1 (By similarity).By similarity2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
GOLGA2Q083793EBI-1021914,EBI-618309
NAA10P412273EBI-1021914,EBI-747693
ZMYND19Q96E353EBI-1021914,EBI-746595

Protein-protein interaction databases

BioGridi123545. 60 interactors.
DIPiDIP-42503N.
IntActiQ6UN15. 45 interactors.
MINTiMINT-1475441.
STRINGi9606.ENSP00000336752.

Structurei

3D structure databases

ProteinModelPortaliQ6UN15.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 356Necessary for stimulating PAPOLA activityAdd BLAST356
Regioni1 – 111Sufficient for interaction with PAPOLAAdd BLAST111
Regioni137 – 243Sufficient for interaction with CPSF41 PublicationAdd BLAST107
Regioni443 – 594Sufficient for interaction with CPSF1 and CSTF31 PublicationAdd BLAST152

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi356 – 406Pro-richAdd BLAST51
Compositional biasi456 – 562Arg-richAdd BLAST107
Compositional biasi478 – 594Glu-richAdd BLAST117

Sequence similaritiesi

Belongs to the FIP1 family.Curated

Phylogenomic databases

eggNOGiKOG1049. Eukaryota.
COG5213. LUCA.
GeneTreeiENSGT00730000111028.
HOGENOMiHOG000004854.
HOVERGENiHBG059889.
KOiK14405.
OMAiGNNIQVI.
PhylomeDBiQ6UN15.
TreeFamiTF318610.

Family and domain databases

InterProiIPR007854. Fip1.
[Graphical view]
PfamiPF05182. Fip1. 1 hit.
[Graphical view]

Sequences (4)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q6UN15-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MSAGEVERLV SELSGGTGGD EEEEWLYGGP WDVHVHSDLA KDLDENEVER
60 70 80 90 100
PEEENASANP PSGIEDETAE NGVPKPKVTE TEDDSDSDSD DDEDDVHVTI
110 120 130 140 150
GDIKTGAPQY GSYGTAPVNL NIKTGGRVYG TTGTKVKGVD LDAPGSINGV
160 170 180 190 200
PLLEVDLDSF EDKPWRKPGA DLSDYFNYGF NEDTWKAYCE KQKRIRMGLE
210 220 230 240 250
VIPVTSTTNK ITAEDCTMEV TPGAEIQDGR FNLFKVQQGR TGNSEKETAL
260 270 280 290 300
PSTKAEFTSP PSLFKTGLPP SRNSTSSQSQ TSTASRKANS SVGKWQDRYG
310 320 330 340 350
RAESPDLRRL PGAIDVIGQT ITISRVEGRR RANENSNIQV LSERSATEVD
360 370 380 390 400
NNFSKPPPFF PPGAPPTHLP PPPFLPPPPT VSTAPPLIPP PGFPPPPGAP
410 420 430 440 450
PPSLIPTIES GHSSGYDSRS ARAFPYGNVA FPHLPGSAPS WPSLVDTSKQ
460 470 480 490 500
WDYYARREKD RDRERDRDRE RDRDRDRERE RTRERERERD HSPTPSVFNS
510 520 530 540 550
DEERYRYREY AERGYERHRA SREKEERHRE RRHREKEETR HKSSRSNSRR
560 570 580 590
RHESEEGDSH RRHKHKKSKR SKEGKEAGSE PAPEQESTEA TPAE
Length:594
Mass (Da):66,526
Last modified:July 5, 2004 - v1
Checksum:iB391D142419ED061
GO
Isoform 3 (identifier: Q6UN15-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     29-43: Missing.
     213-235: Missing.
     272-307: Missing.

Show »
Length:520
Mass (Da):58,376
Checksum:i55D48285A046A783
GO
Isoform 4 (identifier: Q6UN15-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     29-43: Missing.
     393-393: F → K
     394-594: Missing.

Show »
Length:378
Mass (Da):40,835
Checksum:i1B699B114C9560D4
GO
Isoform 5 (identifier: Q6UN15-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     29-43: Missing.
     389-389: P → PPPGIPITVP

Note: No experimental confirmation available.
Show »
Length:588
Mass (Da):65,728
Checksum:i0E9D598DEAF3C752
GO

Sequence cautioni

The sequence AAH24016 differs from that shown. Intron retention.Curated
The sequence AAH52959 differs from that shown. Intron retention.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti118V → I in BAG58575 (PubMed:14702039).Curated1
Sequence conflicti312G → R in AAH24016 (PubMed:15489334).Curated1
Sequence conflicti312G → R in AAH52959 (PubMed:15489334).Curated1
Sequence conflicti524K → R in AAH52959 (PubMed:15489334).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_01672829 – 43Missing in isoform 3, isoform 4 and isoform 5. 3 PublicationsAdd BLAST15
Alternative sequenceiVSP_016729213 – 235Missing in isoform 3. 1 PublicationAdd BLAST23
Alternative sequenceiVSP_016730272 – 307Missing in isoform 3. 1 PublicationAdd BLAST36
Alternative sequenceiVSP_046213389P → PPPGIPITVP in isoform 5. 1 Publication1
Alternative sequenceiVSP_016731393F → K in isoform 4. 1 Publication1
Alternative sequenceiVSP_016732394 – 594Missing in isoform 4. 1 PublicationAdd BLAST201

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY366510 mRNA. Translation: AAQ88277.1.
AL136910 mRNA. Translation: CAB66844.1.
AK295737 mRNA. Translation: BAG58575.1.
AC058822 Genomic DNA. No translation available.
AC095040 Genomic DNA. No translation available.
AC098587 Genomic DNA. No translation available.
AC098821 Genomic DNA. No translation available.
AC105384 Genomic DNA. No translation available.
AC110298 Genomic DNA. No translation available.
AC110792 Genomic DNA. No translation available.
AC124017 Genomic DNA. No translation available.
AC138607 Genomic DNA. No translation available.
AC138779 Genomic DNA. No translation available.
CH471057 Genomic DNA. Translation: EAX05448.1.
BC011543 mRNA. Translation: AAH11543.1.
BC017724 mRNA. Translation: AAH17724.1.
BC024016 mRNA. Translation: AAH24016.1. Sequence problems.
BC052959 mRNA. Translation: AAH52959.1. Sequence problems.
BC110383 mRNA. Translation: AAI10384.1.
AY229892 mRNA. Translation: AAP69563.1. Different termination.
CCDSiCCDS3491.1. [Q6UN15-1]
CCDS47055.1. [Q6UN15-5]
CCDS47056.1. [Q6UN15-3]
RefSeqiNP_001128409.1. NM_001134937.1. [Q6UN15-5]
NP_001128410.1. NM_001134938.1. [Q6UN15-3]
NP_112179.2. NM_030917.3. [Q6UN15-1]
UniGeneiHs.555109.
Hs.624245.

Genome annotation databases

EnsembliENST00000306932; ENSP00000302993; ENSG00000145216. [Q6UN15-3]
ENST00000337488; ENSP00000336752; ENSG00000145216. [Q6UN15-1]
ENST00000358575; ENSP00000351383; ENSG00000145216. [Q6UN15-5]
ENST00000507922; ENSP00000425456; ENSG00000145216. [Q6UN15-4]
GeneIDi81608.
KEGGihsa:81608.
UCSCiuc003gzx.5. human. [Q6UN15-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY366510 mRNA. Translation: AAQ88277.1.
AL136910 mRNA. Translation: CAB66844.1.
AK295737 mRNA. Translation: BAG58575.1.
AC058822 Genomic DNA. No translation available.
AC095040 Genomic DNA. No translation available.
AC098587 Genomic DNA. No translation available.
AC098821 Genomic DNA. No translation available.
AC105384 Genomic DNA. No translation available.
AC110298 Genomic DNA. No translation available.
AC110792 Genomic DNA. No translation available.
AC124017 Genomic DNA. No translation available.
AC138607 Genomic DNA. No translation available.
AC138779 Genomic DNA. No translation available.
CH471057 Genomic DNA. Translation: EAX05448.1.
BC011543 mRNA. Translation: AAH11543.1.
BC017724 mRNA. Translation: AAH17724.1.
BC024016 mRNA. Translation: AAH24016.1. Sequence problems.
BC052959 mRNA. Translation: AAH52959.1. Sequence problems.
BC110383 mRNA. Translation: AAI10384.1.
AY229892 mRNA. Translation: AAP69563.1. Different termination.
CCDSiCCDS3491.1. [Q6UN15-1]
CCDS47055.1. [Q6UN15-5]
CCDS47056.1. [Q6UN15-3]
RefSeqiNP_001128409.1. NM_001134937.1. [Q6UN15-5]
NP_001128410.1. NM_001134938.1. [Q6UN15-3]
NP_112179.2. NM_030917.3. [Q6UN15-1]
UniGeneiHs.555109.
Hs.624245.

3D structure databases

ProteinModelPortaliQ6UN15.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123545. 60 interactors.
DIPiDIP-42503N.
IntActiQ6UN15. 45 interactors.
MINTiMINT-1475441.
STRINGi9606.ENSP00000336752.

PTM databases

iPTMnetiQ6UN15.
PhosphoSitePlusiQ6UN15.

Polymorphism and mutation databases

BioMutaiFIP1L1.
DMDMi74749365.

Proteomic databases

EPDiQ6UN15.
MaxQBiQ6UN15.
PaxDbiQ6UN15.
PeptideAtlasiQ6UN15.
PRIDEiQ6UN15.

Protocols and materials databases

DNASUi81608.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000306932; ENSP00000302993; ENSG00000145216. [Q6UN15-3]
ENST00000337488; ENSP00000336752; ENSG00000145216. [Q6UN15-1]
ENST00000358575; ENSP00000351383; ENSG00000145216. [Q6UN15-5]
ENST00000507922; ENSP00000425456; ENSG00000145216. [Q6UN15-4]
GeneIDi81608.
KEGGihsa:81608.
UCSCiuc003gzx.5. human. [Q6UN15-1]

Organism-specific databases

CTDi81608.
DisGeNETi81608.
GeneCardsiFIP1L1.
HGNCiHGNC:19124. FIP1L1.
HPAiHPA037475.
HPA058202.
MalaCardsiFIP1L1.
MIMi607685. phenotype.
607686. gene.
neXtProtiNX_Q6UN15.
OpenTargetsiENSG00000145216.
Orphaneti520. Acute promyelocytic leukemia.
314950. Primary hypereosinophilic syndrome.
PharmGKBiPA134875694.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1049. Eukaryota.
COG5213. LUCA.
GeneTreeiENSGT00730000111028.
HOGENOMiHOG000004854.
HOVERGENiHBG059889.
KOiK14405.
OMAiGNNIQVI.
PhylomeDBiQ6UN15.
TreeFamiTF318610.

Enzyme and pathway databases

ReactomeiR-HSA-109688. Cleavage of Growing Transcript in the Termination Region.
R-HSA-159231. Transport of Mature mRNA Derived from an Intronless Transcript.
R-HSA-72163. mRNA Splicing - Major Pathway.
R-HSA-72187. mRNA 3'-end processing.
R-HSA-77595. Processing of Intronless Pre-mRNAs.
SIGNORiQ6UN15.

Miscellaneous databases

ChiTaRSiFIP1L1. human.
GeneWikiiFIP1L1.
GenomeRNAii81608.
PMAP-CutDBQ6UN15.
PROiQ6UN15.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000145216.
CleanExiHS_FIP1L1.
ExpressionAtlasiQ6UN15. baseline and differential.
GenevisibleiQ6UN15. HS.

Family and domain databases

InterProiIPR007854. Fip1.
[Graphical view]
PfamiPF05182. Fip1. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiFIP1_HUMAN
AccessioniPrimary (citable) accession number: Q6UN15
Secondary accession number(s): B4DIR3
, G3XAD6, Q0VGE0, Q499Y4, Q49AU3, Q7Z608, Q8WVN3, Q96F80, Q9H077
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 20, 2005
Last sequence update: July 5, 2004
Last modified: November 2, 2016
This is version 124 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.