Atlas of Genetics and Cytogenetics in Oncology and Haematology

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ZMYM2 (fused in myeloproliferative disorders).

Written1998-03Jean-Loup Huret, Dominique Leroux
Lymphoma Research Group - Groupe de Recherche sur les Lymphomes, Institut Albert Bonniot, La Tronche 38706, France (DL)
Updated2001-01Marie-Joséphe Pébusque
INSERM U119, IFR 57, 27 Blvd Lei Roure, 13009 Marseille, France

(Note : for Links provided by Atlas : click)


zinc finger protein 198
Alias_symbol (synonym)RAMP
Other aliasFIM (fused in myeloproliferative disorders).
ZNF198 (zinc finger protein 198).
LocusID (NCBI) 7750
Atlas_Id 114
Location 13q12.11  [Link to chromosome band 13q12]
Location_base_pair Starts at 20532848 and ends at 20665984 bp from pter ( according to hg19-Feb_2009)  [Mapping ZMYM2.png]
Local_order proximal from FLT1 and FLT3
  FIM (13q12) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics.
Fusion genes
(updated 2016)
FGFR1 (8p11.23) / ZMYM2 (13q12.11)ZMYM2 (13q12.11) / DMRT2 (9p24.3)ZMYM2 (13q12.11) / FGFR1 (8p11.23)
ZMYM2 (13q12.11) / MTMR6 (13q12.13)ZMYM2 (13q12.11) / RAB1A (2p14)ZMYM2 (13q12.11) / VWA8 (13q14.11)


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


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

Entity t(8;13)(p12;q12)/AML-NHL --> 5' FIM - 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)
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


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


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 ]
On line version :
History of this paper:
Huret, JL ; Leroux, D. ZNF198 (zinc finger protein 198). Atlas Genet Cytogenet Oncol Haematol. 1998;2(2):52-53.

Other Leukemias implicated (Data extracted from papers in the Atlas) [ 3 ]
  8p11 myeloproliferative syndrome (FGFR1)
t(6;8)(q27;p12) FGFR1OP/FGFR1
t(8;13)(p11;q12) ZMYM2/FGFR1

External links

HGNC (Hugo)ZMYM2   12989
Entrez_Gene (NCBI)ZMYM2  7750  zinc finger MYM-type containing 2
GeneCards (Weizmann)ZMYM2
Ensembl hg19 (Hinxton)ENSG00000121741 [Gene_View]  chr13:20532848-20665984 [Contig_View]  ZMYM2 [Vega]
Ensembl hg38 (Hinxton)ENSG00000121741 [Gene_View]  chr13:20532848-20665984 [Contig_View]  ZMYM2 [Vega]
ICGC DataPortalENSG00000121741
TCGA cBioPortalZMYM2
Genatlas (Paris)ZMYM2
SOURCE (Princeton)ZMYM2
Genetics Home Reference (NIH)ZMYM2
Genomic and cartography
GoldenPath hg19 (UCSC)ZMYM2  -     chr13:20532848-20665984 +  13q11-q12   [Description]    (hg19-Feb_2009)
GoldenPath hg38 (UCSC)ZMYM2  -     13q11-q12   [Description]    (hg38-Dec_2013)
EnsemblZMYM2 - 13q11-q12 [CytoView hg19]  ZMYM2 - 13q11-q12 [CytoView hg38]
Mapping of homologs : NCBIZMYM2 [Mapview hg19]  ZMYM2 [Mapview hg38]
Gene and transcription
Genbank (Entrez)AF012126 AF035374 AF060181 AJ224901 AK226118
RefSeq transcript (Entrez)NM_001190964 NM_001190965 NM_003453 NM_197968
RefSeq genomic (Entrez)NC_000013 NC_018924 NG_023348 NT_024524 NW_004929388
Consensus coding sequences : CCDS (NCBI)ZMYM2
Cluster EST : UnigeneHs.507433 [ NCBI ]
CGAP (NCI)Hs.507433
Alternative Splicing GalleryENSG00000121741
Gene ExpressionZMYM2 [ NCBI-GEO ]   ZMYM2 [ EBI - ARRAY_EXPRESS ]   ZMYM2 [ SEEK ]   ZMYM2 [ MEM ]
Gene Expression Viewer (FireBrowse)ZMYM2 [ Firebrowse - Broad ]
SOURCE (Princeton)Expression in : [Datasets]   [Normal Tissue Atlas]  [carcinoma Classsification]  [NCI60]
GenevisibleExpression in : [tissues]  [cell-lines]  [cancer]  [perturbations]  
BioGPS (Tissue expression)7750
GTEX Portal (Tissue expression)ZMYM2
Protein : pattern, domain, 3D structure
UniProt/SwissProtQ9UBW7   [function]  [subcellular_location]  [family_and_domains]  [pathology_and_biotech]  [ptm_processing]  [expression]  [interaction]
NextProtQ9UBW7  [Sequence]  [Exons]  [Medical]  [Publications]
With graphics : InterProQ9UBW7
Splice isoforms : SwissVarQ9UBW7
Domains : Interpro (EBI)DUF3504    TRASH_dom    Znf_MYM   
Domain families : Pfam (Sanger)DUF3504 (PF12012)    zf-FCS (PF06467)   
Domain families : Pfam (NCBI)pfam12012    pfam06467   
Domain families : Smart (EMBL)TRASH (SM00746)  
Conserved Domain (NCBI)ZMYM2
DMDM Disease mutations7750
Blocks (Seattle)ZMYM2
Human Protein AtlasENSG00000121741
Peptide AtlasQ9UBW7
IPIIPI00646199   IPI00941117   IPI00294603   IPI00640771   
Protein Interaction databases
IntAct (EBI)Q9UBW7
Ontologies - Pathways
Ontology : AmiGORNA polymerase II transcription factor activity, sequence-specific DNA binding  DNA binding  protein tyrosine kinase activity  cytoplasm  cytosol  transcription, DNA-templated  regulation of transcription from RNA polymerase II promoter  cytoskeleton organization  multicellular organism development  biological_process  zinc ion binding  PML body  peptidyl-tyrosine phosphorylation  regulation of cell morphogenesis  ubiquitin conjugating enzyme binding  
Ontology : EGO-EBIRNA polymerase II transcription factor activity, sequence-specific DNA binding  DNA binding  protein tyrosine kinase activity  cytoplasm  cytosol  transcription, DNA-templated  regulation of transcription from RNA polymerase II promoter  cytoskeleton organization  multicellular organism development  biological_process  zinc ion binding  PML body  peptidyl-tyrosine phosphorylation  regulation of cell morphogenesis  ubiquitin conjugating enzyme binding  
REACTOMEQ9UBW7 [protein]
REACTOME Pathways1839117 [pathway]   5655302 [pathway]   
NDEx NetworkZMYM2
Atlas of Cancer Signalling NetworkZMYM2
Wikipedia pathwaysZMYM2
Orthology - Evolution
GeneTree (enSembl)ENSG00000121741
Phylogenetic Trees/Animal Genes : TreeFamZMYM2
Homologs : HomoloGeneZMYM2
Homology/Alignments : Family Browser (UCSC)ZMYM2
Gene fusions - Rearrangements
Fusion : MitelmanZMYM2/DMRT2 [13q12.11/9p24.3]  
Fusion : MitelmanZMYM2/FGFR1 [13q12.11/8p11.23]  [t(8;13)(p11;q12)]  
Fusion : MitelmanZMYM2/MTMR6 [13q12.11/13q12.13]  [t(13;13)(q12;q12)]  
Fusion : MitelmanZMYM2/VWA8 [13q12.11/13q14.11]  [t(13;13)(q12;q14)]  
Fusion: TCGAZMYM2 13q12.11 DMRT2 9p24.3 LUSC
Fusion: TCGAZMYM2 13q12.11 KIAA0564 LUAD
Fusion: TCGAZMYM2 13q12.11 MTMR6 13q12.13 BRCA
Fusion : TICdbZMYM2 [13q12.11]  -  FGFR1 [8p11.23]
Polymorphisms : SNP and Copy number variants
NCBI Variation ViewerZMYM2 [hg38]
dbSNP Single Nucleotide Polymorphism (NCBI)ZMYM2
Exome Variant ServerZMYM2
ExAC (Exome Aggregation Consortium)ZMYM2 (select the gene name)
Genetic variants : HAPMAP7750
Genomic Variants (DGV)ZMYM2 [DGVbeta]
DECIPHER (Syndromes)13:20532848-20665984  ENSG00000121741
CONAN: Copy Number AnalysisZMYM2 
ICGC Data PortalZMYM2 
TCGA Data PortalZMYM2 
Broad Tumor PortalZMYM2
OASIS PortalZMYM2 [ Somatic mutations - Copy number]
Somatic Mutations in Cancer : COSMICZMYM2  [overview]  [genome browser]  [tissue]  [distribution]  
Mutations and Diseases : HGMDZMYM2
intOGen PortalZMYM2
LOVD (Leiden Open Variation Database)Whole genome datasets
LOVD (Leiden Open Variation Database)LOVD - Leiden Open Variation Database
LOVD (Leiden Open Variation Database)LOVD 3.0 shared installation
BioMutasearch ZMYM2
DgiDB (Drug Gene Interaction Database)ZMYM2
DoCM (Curated mutations)ZMYM2 (select the gene name)
CIViC (Clinical Interpretations of Variants in Cancer)ZMYM2 (select a term)
NCG5 (London)ZMYM2
Cancer3DZMYM2(select the gene name)
Impact of mutations[PolyPhen2] [SIFT Human Coding SNP] [Buck Institute : MutDB] [Mutation Assessor] [Mutanalyser]
Genetic Testing Registry ZMYM2
NextProtQ9UBW7 [Medical]
Huge Navigator ZMYM2 [HugePedia]
snp3D : Map Gene to Disease7750
BioCentury BCIQZMYM2
Clinical trials, drugs, therapy
Chemical/Protein Interactions : CTD7750
Chemical/Pharm GKB GenePA37569
Clinical trialZMYM2
canSAR (ICR)ZMYM2 (select the gene name)
PubMed57 Pubmed reference(s) in Entrez
GeneRIFsGene References Into Functions (Entrez)
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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indexed on : Tue Mar 14 13:52:20 CET 2017

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