Written | 2001-01 | Marie-Joséphe Pébusque |
INSERM U119, IFR 57, 27 Blvd Lei Roure, 13009 Marseille, France |
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 |
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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 |
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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) |
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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 ] |
Other Leukemias implicated (Data extracted from papers in the Atlas) [ 8 ] |
External links |
Nomenclature | |
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Cards | |
Atlas | FOPID140.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 | |
Litterature |
REVIEW articles | automatic search in PubMed |
Last year publications | automatic search in PubMed |
© Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Thu Mar 25 20:00:34 CET 2021 |
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