FIP1L1 - factor interacting with PAPOLA and CPSF1 Gene

Also Known as Rhe; FIP1; hFip1

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 81608

About FIP1L1

Cytogenetic location: 4q12 Genomic coordinates (GRCh38): 4:53,377,641-53,460,862 (from NCBI)

This gene has 66 transcripts (splice variants), 29 orthologues and is associated with 70 phenotypes. Ubiquitous expression in testis (RPKM 13.3), brain (RPKM 11.1) and 25 other tissues.

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]

FIP1L1 Products (47)

mRNA Protein Name
NM_001134937.2 NP_001128409.1 pre-mRNA 3'-end-processing factor FIP1 isoform 2
NM_001134938.2 NP_001128410.1 pre-mRNA 3'-end-processing factor FIP1 isoform 3
NM_001376744.1 NP_001363673.1 pre-mRNA 3'-end-processing factor FIP1 isoform 4
NM_001376745.1 NP_001363674.1 pre-mRNA 3'-end-processing factor FIP1 isoform 5
NM_001376746.1 NP_001363675.1 pre-mRNA 3'-end-processing factor FIP1 isoform 6
NM_001376747.1 NP_001363676.1 pre-mRNA 3'-end-processing factor FIP1 isoform 7
NM_001376748.1 NP_001363677.1 pre-mRNA 3'-end-processing factor FIP1 isoform 8
NM_001376749.1 NP_001363678.1 pre-mRNA 3'-end-processing factor FIP1 isoform 9
NM_001376750.1 NP_001363679.1 pre-mRNA 3'-end-processing factor FIP1 isoform 10
NM_001376751.1 NP_001363680.1 pre-mRNA 3'-end-processing factor FIP1 isoform 11
NM_001376752.1 NP_001363681.1 pre-mRNA 3'-end-processing factor FIP1 isoform 12
NM_001376753.1 NP_001363682.1 pre-mRNA 3'-end-processing factor FIP1 isoform 13
NM_001376754.1 NP_001363683.1 pre-mRNA 3'-end-processing factor FIP1 isoform 14
NM_001376755.1 NP_001363684.1 pre-mRNA 3'-end-processing factor FIP1 isoform 15
NM_001376756.1 NP_001363685.1 pre-mRNA 3'-end-processing factor FIP1 isoform 16
NM_001376757.1 NP_001363686.1 pre-mRNA 3'-end-processing factor FIP1 isoform 17
NM_001376758.1 NP_001363687.1 pre-mRNA 3'-end-processing factor FIP1 isoform 18
NM_001376759.1 NP_001363688.1 pre-mRNA 3'-end-processing factor FIP1 isoform 19
NM_001376760.1 NP_001363689.1 pre-mRNA 3'-end-processing factor FIP1 isoform 20
NM_001376761.1 NP_001363690.1 pre-mRNA 3'-end-processing factor FIP1 isoform 21
NM_001376762.1 NP_001363691.1 pre-mRNA 3'-end-processing factor FIP1 isoform 22
NM_001376764.1 NP_001363693.1 pre-mRNA 3'-end-processing factor FIP1 isoform 23
NM_001376765.1 NP_001363694.1 pre-mRNA 3'-end-processing factor FIP1 isoform 24
NM_001376766.1 NP_001363695.1 pre-mRNA 3'-end-processing factor FIP1 isoform 25
NM_001376767.1 NP_001363696.1 pre-mRNA 3'-end-processing factor FIP1 isoform 26
NM_001376768.1 NP_001363697.1 pre-mRNA 3'-end-processing factor FIP1 isoform 27
NM_001376769.1 NP_001363698.1 pre-mRNA 3'-end-processing factor FIP1 isoform 28
NM_001376770.1 NP_001363699.1 pre-mRNA 3'-end-processing factor FIP1 isoform 29
NM_001376771.1 NP_001363700.1 pre-mRNA 3'-end-processing factor FIP1 isoform 30
NM_001376772.1 NP_001363701.1 pre-mRNA 3'-end-processing factor FIP1 isoform 31
NM_001376773.1 NP_001363702.1 pre-mRNA 3'-end-processing factor FIP1 isoform 32
NM_001376774.1 NP_001363703.1 pre-mRNA 3'-end-processing factor FIP1 isoform 33
NM_001376775.1 NP_001363704.1 pre-mRNA 3'-end-processing factor FIP1 isoform 34
NM_001376776.1 NP_001363705.1 pre-mRNA 3'-end-processing factor FIP1 isoform 35
NM_001376777.1 NP_001363706.1 pre-mRNA 3'-end-processing factor FIP1 isoform 36
NM_001376778.1 NP_001363707.1 pre-mRNA 3'-end-processing factor FIP1 isoform 37
NM_001376779.1 NP_001363708.1 pre-mRNA 3'-end-processing factor FIP1 isoform 38
NM_001376780.1 NP_001363709.1 pre-mRNA 3'-end-processing factor FIP1 isoform 39
NM_001376781.1 NP_001363710.1 pre-mRNA 3'-end-processing factor FIP1 isoform 40
NM_001376782.1 NP_001363711.1 pre-mRNA 3'-end-processing factor FIP1 isoform 41
NM_001376783.1 NP_001363712.1 pre-mRNA 3'-end-processing factor FIP1 isoform 42
NM_001376784.1 NP_001363713.1 pre-mRNA 3'-end-processing factor FIP1 isoform 43
NM_001376785.1 NP_001363714.1 pre-mRNA 3'-end-processing factor FIP1 isoform 44
NM_001376786.1 NP_001363715.1 pre-mRNA 3'-end-processing factor FIP1 isoform 45
NM_001410723.1 NP_001397652.1 pre-mRNA 3'-end-processing factor FIP1 isoform 46
NM_001410724.1 NP_001397653.1 pre-mRNA 3'-end-processing factor FIP1 isoform 47
NM_030917.4 NP_112179.2 pre-mRNA 3'-end-processing factor FIP1 isoform 1
Molecular Function GO Annotation Evidence References Source
enables protein binding IPI
IPI: Inferred from physical interaction
15937220 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

FIP1L1 Protein Structure

Fip1

Fip1: Fip1 motif (139 - 182)

  • 0
  • 100
  • 200
  • 300
  • 400
  • 500
  • 588 a.a.
Protein Preferred Names Protein Names

pre-mRNA 3'-end-processing factor FIP1

  • FIP1 like 1

FIP1L1 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
FIP1L1 Q6UN15 GOLGA2 Homo sapiens Q08379 25416956
Intra
FIP1L1 Q6UN15 CPSF4 Homo sapiens O95639 26496610
Intra
FIP1L1 Q6UN15 NAA10 Homo sapiens P41227 25416956
Intra
FIP1L1 Q6UN15 NAA10 Homo sapiens P41227 25416956
Intra
FIP1L1 Q6UN15 NAA10 Homo sapiens P41227 25416956
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Chronic Eosinophilic Leukemia
  • Pdgfra-Associated Chronic Eosinophilic Leukemia

Primary Hypereosinophilic Syndrome
  • Clonal Hypereosinophilic Syndrome

  • Hes-M

  • Hes-N

  • Neoplastic Hypereosinophilic Syndrome

  • Primary Hes

  • Hm Syndrome

Acute Promyelocytic Leukemia
  • Leukemia, Acute Promyelocytic

  • Acute Myeloblastic Leukemia Type 3

  • Aml M3

  • APL

  • Leukemia, Acute Promyelocytic, Somatic

  • Aml With T(15

  • 17)(Q22

  • Q12)

  • (Pml/Raralpha) And Variants

  • Apml

  • Acute Myeloblastic Leukemia 3

  • Acute Myeloid Leukemia With T(15

  • 17)(Q22

  • Q12)

  • (Pml/Raralpha) And Variants

  • Acute Myeloblastic Leukaemia Type 3

  • Acute Myeloid Leukaemia M3

  • Acute Myeloid Leukemia M3

  • Acute Promyelocytic Leukaemia

  • M3 Anll

  • Myeloid Leukemia, Acute, M3

  • Leukemia Promyelocytic Acute

  • Leukemia, Promyelocytic, Acute

  • Leukemia, Acute, Promyelocytic

Hypereosinophilic Syndrome
  • Eosinophilia

  • HES

  • Eosinophilic Leukocytosis

  • Hypereosinophilic Syndrome, Idiopathic

  • Idiopathic Hypereosinophilic Syndrome

  • Disseminated Eosinophilic Collagen Disease

  • Eosinophilic Disorder

  • Disorders With Increased Eosinophil Counts

Hypereosinophilic Syndrome, Idiopathic
  • Idiopathic Hypereosinophilic Syndrome

  • Hypereosinophilic Syndrome, Idiopathic, Resistant To Imatinib

  • HES

Mastocytosis, Cutaneous
  • Urticaria Pigmentosa

  • Cutaneous Mastocytosis

  • Diffuse Cutaneous Mastocytosis

  • Maculopapular Cutaneous Mastocytosis

  • Mastocytosis, Diffuse Cutaneous

  • MASTC

  • Mastocytosis, Maculopapular Cutaneous

  • Mastocytosis, Systemic, Somatic

  • Mastocytoma

  • Dcm

  • Diffuse Cutaneous Maculopapulous Mastocytosis

  • Up/Mpcm

  • Cm

  • Mastocytosis, Systemic

  • MASTSYS

  • Mast Cell Disease

  • Mast-Cell Disease

  • Mast Cell Leukemia

  • Mastocytosis, Indolent

  • Mastocytosis With Associated Hematologic Disorder

  • Mast Cell Neoplasm

  • Benign Mastocytoma

Loeffler Endocarditis
  • Eosinophilic Endomyocardial Disease

  • Eosinophilic Endocarditis

  • Loeffler'S Endocarditis

  • Primary Eosinophilic Endomyocardial Restrictive Cardiomyopathy

Stress Polycythemia
  • Gaisbock'S Syndrome

  • Polycythemia, Emotional

  • Pseudopolycythaemia

  • Gaisboeck'S Syndrome

  • Pseudopolycythemia

  • Gaisbock Syndrome

  • Stress Erythrocytosis

  • Pseudo-Polycythemia

  • Relative Erythrocytosis

Systemic Mastocytosis
  • Systemic Mast Cell Disease

  • Systemic Tissue Mast Cell Disease

  • Mastocytosis, Systemic

  • Smcd - Systemic Mast Cell Disease

  • Smcd

  • Mast Cell Disease, Systemic

  • Systemic Mast-Cell Disease

  • Systemic Mastocytoses

  • Mastocytosis Systemic

  • Corneal Dystrophy, Subepithelial Mucinous

  • Smcd - [Systemic Mast Cell Disease]

Myeloid And Lymphoid Neoplasms With Eosinophilia And Abnormalities Of Pdgfra, Pdgfrb, And Fgfr1
  • Myeloid And Lymphoid Neoplasms With Eosinophilia And Abnormalities Of Platelet-Derived Growth Factor Receptor Alpha , Platelet-Derived Growth Factor Receptor Beta , And Fibroblast Growth Factor Receptor-1 Are A Group Of Hematologic Neoplasms

  • Myeloid And Lymphoid Neoplasms With Eosinophilia And Rearrangement Of Pdgfra, Pdgfrb, Or Fgfr1, Or With Pcm1-Jak2

Mastocytosis
  • Mast Cell Disease

  • Mast Cell Hyperplasia

  • Urticaria Pigmentosa

  • Malignant Mastocytoma

  • Malignant Mast Cell Tumours

  • Malignant Mastocytosis

Budd-Chiari Syndrome
  • Hepatic Vein Thrombosis

  • Chiari Syndrome

  • BDCHS

  • Membranous Obstruction Of The Inferior Vena Cava

  • Budd-Chiari Syndrome, Somatic

  • Movc

  • Budd-Chiari Syndrome, Susceptibility To, Somatic

  • Budd-Chiari Syndrome, Susceptibility To

  • Membranous Obstruction Of Inferior Vena Cava

  • Hepatic Vein Block

  • Obstruction Of Hepatic Veins

  • Hepatic Vein Obstruction

  • Hepatic Venous Block

Disseminated Eosinophilic Collagen Disease
Sm-Ahnmd
  • Systemic Mastocytosis With Associated Clonal, Hematologic Non-Mast-Cell Lineage Disease

  • Systemic Mastocytosis With Associated Clonal Haematological Non-Mast Cell Lineage Disease

  • Systemic Mastocytosis With Associated Clonal Hematological Non-Mast-Cell Lineage Disease

Endomyocardial Fibrosis
  • Becker'S Disease

  • Obscure African Cardiomyopathy

  • African Endomyocardial Fibrosis

  • Endomyocardial Sclerosis

  • EMF

  • Becker Muscular Dystrophy

Myeloid And Lymphoid Neoplasms Associated With Pdgfra Rearrangement
  • Myeloid/Lymphoid Neoplasm Associated With Pdgfra Rearrangement

  • Myeloid And Lymphoid Neoplasms With Pdgfra Rearrangement

Toxocariasis
  • Toxocara Infection

  • Visceral Larva Migrans

  • Larva Migrans, Visceral

  • Infection By Toxascaris

  • Larva Migrans Visceral

  • Larva Migrans Visceralis

  • Toxocaral Visceral Larva Migrans

  • Visceral Larva Migrans Syndrome

  • Toxocara Infestation

Extracutaneous Mastocytoma
  • Mastocytoma

  • Mast Cell Neoplasm

  • Benign Mastocytoma

Indolent Systemic Mastocytosis
  • Ism

Myeloproliferative Disorder, Chronic, With Eosinophilia
  • Myeloproliferative Disorder With Eosinophilia

  • MPE

  • Emp

  • Eosinophils, Malignant Proliferation Of

  • Chronic Myeloproliferative Disorder With Eosinophilia

  • Chronic Myeloproliferative Disease, Unclassifiable

  • Cmpd-U

  • Undifferentiated Myeloproliferative Disease

  • Myeloproliferative Disorder Chronic With Eosinophilia

  • Malignant Proliferation Of Eosinophils

  • Myeloproliferative Neoplasm, Unclassifiable

Chronic Eosinophilic Pneumonia
  • Carrington Syndrome

  • Idiopathic Chronic Eosinophilic Pneumonia

  • Cryptogenic Pulmonary Eosinophilia

  • Idiopathic Eosinophilic Chronic Pneumopathy

  • Carrington'S Disease

  • Carrington'S Pulmonary Eosinophilia

  • Chronic Idiopathic Eosinophilic Pneumonia

  • Eosinophilic Idiopathic Chronic Pneumopathy

  • Eosinophilic Pneumonia Chronic

Chronic Leukemia
  • Adult Chronic Leukemia

  • Cll

  • Cml

Loeffler Syndrome
  • Loeffler'S Pneumonia

  • Loffler'S Syndrome

  • Simple Pulmonary Eosinophilia

Aggressive Systemic Mastocytosis
  • Asm

  • Lymphadenopathic Mastocytosis With Eosinophilia

Mast-Cell Sarcoma
  • Mast Cell Sarcoma

Polycythemia Vera
  • PV

  • Polycythemia Rubra Vera

  • Prv

  • Osler-Vaquez Disease

  • Chronic Erythremia

  • Polycythaemia Rubra Vera

  • Primary Polycythemia

  • Vaquez Disease

  • Polycythemia Vera, Somatic

  • Osler-Vaquez Syndrome

  • Proliferative Polycythaemia

  • Polycythemia Ruba Vera

  • Acquired Primary Erythrocytosis

  • Heilmeyer-Schoner Disease

  • Vaquez Osler Disease

  • Primary Polycythaemia

Eosinophilic Gastroenteritis
  • Eosinophilic Enteritis

  • Ege

  • Eosinophilic Gastroenterocolitis

  • Eosinophilic Esophagitis

  • Eosinophilic Gastritis

  • Eosinophilic Gastroenteropathy

  • Gastroenteritis, Eosinophilic

  • Eosinophilic Enteropathy

Eosinophilic Pneumonia
  • Pneumonia, Eosinophilic

  • Pulmonary Eosinophilia

  • Asthmatic Pulmonary Eosinophilia

  • Pneumonia Eosinophilic

  • Simple Pulmonary Eosinophilia

  • Eosinophilic Asthma

Sarcocystosis
  • Sarcosporidiosis

  • Sarcosporidiasis

Eosinophilic Gastritis
  • Eosinophilic Enteropathy

Eosinophilia-Myalgia Syndrome
  • Eosinophilia Myalgia Syndrome

  • Ems

  • L-Tryptophan Induced Ems

  • Severe Muscle Pain And Abnormally High Eosinophils

  • Syndrome With Inflammatory And Autoimmune Components That Affect The Skin, Fascia, Muscle, Nerve, Blood Vessels, Lung, And Heart

Mononeuritis Multiplex
Restrictive Cardiomyopathy
  • Familial Restrictive Cardiomyopathy

  • Cardiomyopathy, Restrictive

  • Cardiomyopathy, Constrictive

  • Primary Restrictive Cardiomyopathy

  • Rcm

  • Cardiomyopathy Restrictive

Corneal Dystrophy, Subepithelial Mucinous
  • Subepithelial Mucinous Corneal Dystrophy

  • SMCD

Leukocyte Disease
  • Leukocyte Disorders

Granulomatosis With Polyangiitis
  • GPA

  • Wegener Granulomatosis

  • Wegener Granulomatosis, Formerly

  • Midline Granulomatosis

  • Wg, Formerly

  • Necrotizing Respiratory Granulomatosis

  • Wg

  • Wegeners Granulomatosis

  • Pauci-Immune Glomerulonephritis Associated With Granulomatosis With Polyangiitis

Chronic Myelomonocytic Leukemia
  • Leukemia, Myelomonocytic, Chronic

  • Cmml

  • Leukemia Myelomonocytic Chronic

  • Cmml - [Chronic Myelomonocytic Leukaemia]

  • Chronic Myelomonocytic Leukaemia Without Mention Of Remission

  • Chronic Monocytic Leukaemia

  • Chronic Monocytoid Leukaemia

Leukemia, Acute Myeloid
  • Acute Myeloid Leukemia

  • Leukemia, Acute Myelogenous

  • Acute Myelogenous Leukemia

  • AML

  • Leukemia, Acute Myeloid, Susceptibility To

  • Acute Myeloblastic Leukemia

  • Leukemia, Acute Myeloid, Reduced Survival In, Somatic

  • Acute Myeloid Leukaemia

  • Leukemia, Myelocytic, Acute

  • Therapy Related Acute Myeloid Leukemia And Myelodysplastic Syndrome

  • Secondary Aml

  • Acute Myelocytic Leukemia

  • Acute Myeloid Leukemia, Somatic

  • Leukemia, Acute Myeloid, Somatic

  • Myeloid Leukemia, Acute, M4/M4eo Subtype, Somatic

  • Acute Myeloblastic Leukaemia

  • Acute Myelogenous Leukaemia

  • Aml - Acute Myeloid Leukemia

  • Acute Myeloid Leukemia With Cebpa Somatic Mutations

  • Aml With Cebpa Somatic Mutations

  • Inherited Acute Myeloid Leukemia

  • Familial Aml

  • Inherited Aml

  • Pure Familial Aml

  • Pure Familial Acute Myeloid Leukemia

  • Secondary Acute Myeloid Leukemia

  • Therapy-Related Aml And Myelodysplastic Syndrome

  • Acute Myeloid Leukemia, Secondary

  • Acute Non-Lymphoblastic Leukemia

  • Acute Non-Lymphocytic Leukemia

  • Acute Biphenotypic Leukemia

  • Acute Undifferentiated Leukemia

  • Acute Myeloblastic Leukaemia With Multilineage Dysplasia

  • Acute Myeloid Leukaemia With Multilineage Dysplasia Without Mention Of Remission

  • Acute Myeloid Leukaemia With Myelodysplasia-Related Features

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Mus musculus FIP1L1 MGD MGI:1914149
Felis catus FIP1L1 VGNC VGNC:78498
Bos taurus FIP1L1 VGNC VGNC:53714
Canis familiaris FIP1L1 VGNC VGNC:40883
Rattus norvegicus FIP1L1 RGD RGD:1309336
Others FIP1L1 NCBI