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ZMYM2 (fused in myeloproliferative disorders).

Written2001-01Marie-Joséphe Pébusque
INSERM U119, IFR 57, 27 Blvd Lei Roure, 13009 Marseille, France
This article is an update of :
1998-03Jean-Loup Huret, Dominique Leroux
Lymphoma Research Group - Groupe de Recherche sur les Lymphomes, Institut Albert Bonniot, La Tronche 38706, France (DL)

(Note : for Links provided by Atlas : click)

Identity

Alias (NCBI)FIM (fused in myeloproliferative disorders).
ZNF198 (zinc finger protein 198).
RAMP
HGNC (Hugo) ZMYM2
HGNC Alias symbRAMP
FIM
MYM
HGNC Previous nameZNF198
HGNC Previous namezinc finger protein 198
LocusID (NCBI) 7750
Atlas_Id 114
Location 13q12.11  [Link to chromosome band 13q12]
Location_base_pair Starts at 19958727 and ends at 20089115 bp from pter ( according to GRCh38/hg38-Dec_2013)  [Mapping ZMYM2.png]
Local_order proximal from FLT1 and FLT3
 
  FIM (13q12) - 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)::ZMYM2 (13q12.11)ZMYM2 (13q12.11)::DMRT2 (9p24.3)ZMYM2 (13q12.11)::FGFR1 (8p11.23)
ZMYM2 (13q12.11)::FLT3 (13q12.2)ZMYM2 (13q12.11)::MTMR6 (13q12.13)ZMYM2 (13q12.11)::RAB1A (2p14)
ZMYM2 (13q12.11)::VWA8 (13q14.11)

DNA/RNA

Description full length cDNA: 5,016 bp; a single open reading frame of 4,137 bp; alternative spliced cDNA variant
Transcription main transcripts: 5.0 and 7.5 kb

Protein

 
Description 1 379 amino acids; hydrophobic protein containing several motifs: a N-terminal cystein-rich region containing ten repeats with the consensus sequence C-X2-C-X18-24-F/Y-C-X3-C, which correspond to a novel zinc finger motifs, a highly hydrophobic proline-rich stretch, and a bipartite nuclear localization signal
Expression wide
Localisation cell nucleus and nucleolus; within the nucleolus, colocalizes with UBF (Upstream Binding Factor)
Function may be involved in the regulation of rRNA transcription
Homology FIM is related to DXS6673E, a gene which may be related with mental retardation

Implicated in

Note
  
Entity /AML-NHL --> 5' ZMYM2 - 3' FGFR1 ; stem-cell myeloproliferative disorder associated with the 8p12 chromosomal translocations; fused 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 usually, t(8;13)(p12;q12) occurs as a single anomaly; duplication of the der(13) was found during disease progression, suggesting that the crucial event might lie on this derivative chromosome; additional abnormalities:+8, +21
Hybrid/Mutated Gene 5' FIM - 3' FGFR1; localisation: der(13)
 
DNA Diagram
Abnormal Protein aberrant tyrosine kinase composed of the N-term two-thirds of FIM (retaining the 10 putative zinc finger motifs), and the 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 constitutive dimerization capability mediated by the FIM N-term zinc finger sequences
  

Bibliography

Characterization of FIM-FGFR1, the fusion product of the myeloproliferative disorder-associated t(8;13) translocation.
Ollendorff V, Guasch G, Isnardon D, Galindo R, Birnbaum D, Pébusque MJ
The Journal of biological chemistry. 1999 ; 274 (38) : 26922-26930.
PMID 10480903
 
Fibroblast growth factor receptor 1 is fused to FIM in stem-cell myeloproliferative disorder with t(8;13).
Popovici C, Adélaïde J, Ollendorff V, Chaffanet M, Guasch G, Jacrot M, Leroux D, Birnbaum D, Pébusque MJ
Proceedings of the National Academy of Sciences of the United States of America. 1998 ; 95 (10) : 5712-5717.
PMID 9576949
 
The t(8;13)(p11;q11-12) rearrangement associated with an atypical myeloproliferative disorder fuses the fibroblast growth factor receptor 1 gene to a novel gene RAMP.
Smedley D, Hamoudi R, Clark J, Warren W, Abdul-Rauf M, Somers G, Venter D, Fagan K, Cooper C, Shipley J
Human molecular genetics. 1998 ; 7 (4) : 637-642.
PMID 9499416
 
ZNF198-FGFR1 transforming activity depends on a novel proline-rich ZNF198 oligomerization domain.
Xiao S, McCarthy JG, Aster JC, Fletcher JA
Blood. 2000 ; 96 (2) : 699-704.
PMID 10887137
 
FGFR1 is fused with a novel zinc-finger gene, ZNF198, in the t(8;13) leukaemia/lymphoma syndrome.
Xiao S, Nalabolu SR, Aster JC, Ma J, Abruzzo L, Jaffe ES, Stone R, Weissman SM, Hudson TJ, Fletcher JA
Nature genetics. 1998 ; 18 (1) : 84-87.
PMID 9425908
 

Citation

This paper should be referenced as such :
Pébusque, MJ
FIM (fused in myeloproliferative disorders).
Atlas Genet Cytogenet Oncol Haematol. 2001;5(1):27-28.
Free journal version : [ pdf ]   [ DOI ]
History of this paper:
Huret, JL ; Leroux, D. ZNF198 (zinc finger protein 198). Atlas Genet Cytogenet Oncol Haematol. 1998;2(2):52-53.
http://documents.irevues.inist.fr/bitstream/handle/2042/37413/03-1998-ZNF198ID114.pdf


Other Leukemias implicated (Data extracted from papers in the Atlas) [ 4 ]
  8p11 myeloproliferative syndrome (FGFR1)
t(6;8)(q27;p12) FGFR1OP::FGFR1
t(8;13)(p11;q12) ZMYM2::FGFR1
t(13;13)(q12;q12) ZMYM2::FLT3


External links

Nomenclature
HGNC (Hugo)ZMYM2   12989
Cards
AtlasZNF198ID114
Atlas Explorer : (Salamanque)ZMYM2
Entrez_Gene (NCBI)ZMYM2    zinc finger MYM-type containing 2
AliasesFIM; MYM; NECRC; RAMP; 
SCLL; ZNF198
GeneCards (Weizmann)ZMYM2
Ensembl hg19 (Hinxton)ENSG00000121741 [Gene_View]
Ensembl hg38 (Hinxton)ENSG00000121741 [Gene_View]  ENSG00000121741 [Sequence]  chr13:19958727-20089115 [Contig_View]  ZMYM2 [Vega]
ICGC DataPortalENSG00000121741
TCGA cBioPortalZMYM2
AceView (NCBI)ZMYM2
Genatlas (Paris)ZMYM2
SOURCE (Princeton)ZMYM2
Genetics Home Reference (NIH)ZMYM2
Genomic and cartography
GoldenPath hg38 (UCSC)ZMYM2  -     chr13:19958727-20089115 +  13q12.11   [Description]    (hg38-Dec_2013)
GoldenPath hg19 (UCSC)ZMYM2  -     13q12.11   [Description]    (hg19-Feb_2009)
GoldenPathZMYM2 - 13q12.11 [CytoView hg19]  ZMYM2 - 13q12.11 [CytoView hg38]
ImmunoBaseENSG00000121741
Genome Data Viewer NCBIZMYM2 [Mapview hg19]  
OMIM602221   619522   
Gene and transcription
Genbank (Entrez)AF012126 AF035374 AF060181 AJ224901 AK226118
RefSeq transcript (Entrez)NM_001190964 NM_001190965 NM_001353157 NM_001353159 NM_001353161 NM_001353162 NM_001353163 NM_001353164 NM_001353165 NM_003453 NM_197968
Consensus coding sequences : CCDS (NCBI)ZMYM2
Gene ExpressionZMYM2 [ NCBI-GEO ]   ZMYM2 [ EBI - ARRAY_EXPRESS ]   ZMYM2 [ SEEK ]   ZMYM2 [ MEM ]
Gene Expression Viewer (FireBrowse)ZMYM2 [ Firebrowse - Broad ]
GenevisibleExpression of ZMYM2 in : [tissues]  [cell-lines]  [cancer]  [perturbations]  
BioGPS (Tissue expression)7750
GTEX Portal (Tissue expression)ZMYM2
Human Protein AtlasENSG00000121741-ZMYM2 [pathology]   [cell]   [tissue]
Protein : pattern, domain, 3D structure
Domain families : Pfam (Sanger)
Domain families : Pfam (NCBI)
Conserved Domain (NCBI)ZMYM2
Human Protein Atlas [tissue]ENSG00000121741-ZMYM2 [tissue]
HPRD03745
Protein Interaction databases
BioGRIDZMYM2
STRING (EMBL)ZMYM2
ZODIACZMYM2
Ontologies - Pathways
Litterature
PubMed97 Pubmed reference(s) in Entrez
GeneRIFsGene References Into Functions (Entrez)
EVEXZMYM2
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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