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FGFR1OP (FGFR1 oncogene partner)

Written2001-01Marie-Joséphe Pébusque
INSERM U119, IFR 57, 27 Blvd Lei Roure, 13009 Marseille, France

(Note : for Links provided by Atlas : click)

Identity

Alias (NCBI)FOP (Fibroblast Growth Factor Receptor 1 Oncogene Partner)
LocusID (NCBI) 11116
Atlas_Id 140
Location 6q27  [Link to chromosome band 6q27]
Location_base_pair Starts at and ends at bp from pter
 
  FOP (6q27) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics.
Fusion genes
(updated 2017)
Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands)
FGFR1 (8p11.23) / FGFR1OP (6q27)FGFR1OP (6q27) / ABCC4 (13q32.1)FGFR1OP (6q27) / FGFR1 (8p11.23)
FGFR1OP (6q27) / MCM9 (6q22.31)FGFR1OP (6q27) / RET (10q11.21)TARS (5p13.3) / FGFR1OP (6q27)

DNA/RNA

Description full length cDNA: 1 627 bp
Transcription a single open reading frame of 1 197 bp mRNA; putative ATG: bp 85; stop codon at bp 1 282; alternative splicing: multiple FOP transcript variants resulting from exon 7 or exon 11 splices

Protein

 
Description 399 amino acids; predicted molecular mass: 44.3 kDa; Hydrophobic protein containing in its N- and C-termini several regions folding in a-helices with leucine-rich repeats with the consensus sequence L-X2-L-X3-5-L-X3-5-L, in one-third of which the leucine is substituted by either a valine or an isoleucine
Expression ubiquitous expression
Localisation cell cytoplasm
Function unknown

Implicated in

Note
  
Entity t(6;8)(q27; p12) myeloproliferative disorder --> FGFR1OP - FGFR1 ; stem-cell myeloproliferative disorder associated with the 8p12 chromosomal translocations with fusions to the catalytic domain of FGFR1
Disease stem-cell myeloproliferative disorder characterized by myeloid hyperplasia, T -cell lymphoblastic leukemia/lymphoma and peripheral blood eosinophilia, and it generally progresses to acute myeloid leukemia; specific to the 8p12 chromosomal region.
Prognosis very poor (median survival: 12 mths)
Cytogenetics additional abnormalities: 2q+ and +21
Hybrid/Mutated Gene 5' FOP - 3' FGFR1; localisation: der(6)
Abnormal Protein N-term leucine-rich region from FOP fused to the catalytic domain of FGFR1 (FGFR1 intracellular region minus the major part of the juxtamembrane domain)
 
Oncogenesis constitutive kinase activity of FGFR1 through constitutive activation of FGFR1 signal transduction pathways via putative constitutive dimerization capability mediated by the FOP N-term LRR sequences
  

Bibliography

The t(6;8)(q27;p11) translocation in a stem cell myeloproliferative disorder fuses a novel gene, FOP, to fibroblast growth factor receptor 1.
Popovici C, Zhang B, Grégoire MJ, Jonveaux P, Lafage-Pochitaloff M, Birnbaum D, Pébusque MJ
Blood. 1999 ; 93 (4) : 1381-1389.
PMID 9949182
 

Citation

This paper should be referenced as such :
Pébusque, MJ
FGFR1OP (FGFR1 oncogene partner)
Atlas Genet Cytogenet Oncol Haematol. 2001;5(1):21-22.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Genes/FOPID140.html



Other Leukemias implicated (Data extracted from papers in the Atlas) [ 8 ]
  8p11 myeloproliferative syndrome (FGFR1)
t(6;8)(q27;p12) FGFR1OP/FGFR1
t(6;10)(q27;q11) FGFR1OP/RET
t(6;10)(q27;q11) FGFR1OP/RET
8p11 myeloproliferative syndrome (FGFR1)
t(6;8)(q27;p12) FGFR1OP/FGFR1
t(6;10)(q27;q11) FGFR1OP/RET
t(6;10)(q27;q11) FGFR1OP/RET



Other Solid tumors implicated (Data extracted from papers in the Atlas) [ 4 ]
  t(6;6)(q22;q27) FGFR1OP/MCM9
t(6;13)(q27;q32) FGFR1OP/ABCC4
t(6;6)(q22;q27) FGFR1OP/MCM9
t(6;13)(q27;q32) FGFR1OP/ABCC4


External links

Nomenclature
Cards
AtlasFOPID140.txt
Aliases
Genomic and cartography
Gene and transcription
RefSeq transcript (Entrez)
RefSeq genomic (Entrez)
BioGPS (Tissue expression)11116
Protein : pattern, domain, 3D structure
Domain families : Pfam (Sanger)
Domain families : Pfam (NCBI)
Protein Interaction databases
Ontologies - Pathways
Clinical trials, drugs, therapy
Miscellaneous
canSAR (ICR) (select the gene name)
Probes
Litterature
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed


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indexed on : Thu Jul 16 16:44:05 CEST 2020

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