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FCGR2B (Fc fragment of IgG, low affinity IIb, receptor (CD32))

Written2002-02Mary Callanan, Dominique Leroux
Lymphoma Research Group - Groupe de Recherche sur les Lymphomes - EMI0353, Institut Albert Bonniot, Université Joseph Fourier Grenoble 1, La Tronche 38706, France

(Note : for Links provided by Atlas : click)

Identity

Other aliasCD32
FCG2
FCGR2
IGFR2
LocusID (NCBI) 2213
Atlas_Id 397
Location 1q23.3  [Link to chromosome band 1q23]
Location_base_pair Starts at and ends at bp from pter
Local_order location, by FISH/genome sequencing; FCGR2B is contained within a locus that spans approximately 200kb and that encodes 5 low affinity IgG Fc receptor genes.The published gene orientation is as follows; Cen 1q -- 3' FCGR2A 5' -- 5'- FCGR3A - 3' -- 3' FCGR2B 5' -- 5' - FCGR3B - 3' -- 5'- FCGR2C- 3'.
 
  Probe(s) - 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)
FCGR2B (1q23.3) / CFAP97 (4q35.1)STPG1 (1p36.11) / FCGR2B (1q23.3)

DNA/RNA

Description The FCGR2B gene spans a 15kb genomic region; Gene orientation : centromere - 3' FCGR2B 5' - telomere; 8 exons (denoted S1, S2, EC1, EC2, TM, IC1, IC2, IC3). S1 and S2 encode the signal peptide domain, EC1 and EC2 encode the extracellular domain, TM encodes the transmembrane domain and IC1, IC2, IC3 encode the intracytoplasmic region.
Transcription 1.7kb mRNA. Two alternately spliced mRNA exist in humans; FcgRIIb1 and FcgRIIb2 (a 19 aa insertion encoded by IC1 is present in b1 but not in b2).

Protein

Note called Fc gamma RIIB
Description 40kD transmembrane glycoprotein that possesses an intracytoplasmic Immunoreceptor Tyrosine-based Inhibition Motif (ITIM) (encoded by IC3).
Expression B, myeloid and mast cells.
Localisation Transmembrane.
Function Low affinity IgG Fc receptor. Founding member ITIM family of immune inhibitory receptors. Fc gamma RIIB functions to down-regulate activatory responses mediated by Immunoreceptor Tyrosine-based Inhibition Motif (ITAM) bearing receptors such as the B-cell receptor on B cells. For example, on B-cells, negative regulation of B-cell receptor activatory signalling is initiated through co-recruitment of BCR and Fc gamma RIIB to IgG/ immune complexes. This leads to tyrosyl phosphorylation of the Fc gamma RIIB ITIM motif / recruitment of the inositol phosphatase SHIP and activation of the adaptor protein Dok - these events ultimately lead to the inhibition of ITAM-dependant Ca++ mobilisation and cellular proliferation (reduced MAPK signalling), respectively.
A proapoptotic signal through the Fc gamma RIIB transmembrane sequence has been described in conditions of homo-aggregation of Fc gamma RII on murine B cells.
Homology Contains two immunoglobulin-like C2 domains.

Implicated in

Note
  
Entity 1q21-23 rearrangements in NHL (Nnon-Hodgkins Lymphoma)
Disease NHL (< 5% of cases with 1q21-23 breaks)
Prognosis It is now known that cytogenetically determined 1q21-23 breaks can target a diversity of 1q21-23 genes. Early data has suggested a poor prognosis for 1q21 rearrangements in diffuse large cell lymphoma.
Cytogenetics Deregulation of the FCGR2B gene by chromosomal translocation was first demonstrated in 3 NHL patients that showed a t(1;22)(q22;q22) in association with a t(14;18)(q32;q21). Two further cases of NHL with rearrangements affecting the FCGR2B region have since been identified. Both cases also showed t(14;18)(see below for description). This suggests a role for this deregulation in lymphoma progression.
Hybrid/Mutated Gene Dysregulation of the FCGR2B gene has been identified as a consequence of a t(1;22)(q22;q11) (3 patients) and t(1;14)(q21;q32) (1 patient). The FCGR2B coding sequence and promoter region appear to remain intact in these translocations. The principal consequence is overexpression of the Fc gamma RIIb2 isoform of Fc gamma RIIB. Deregulation of FCGR2B most likely occurs as a consequence of Ig gene transcriptional enhancer activity. Other mechanisms may exist ; an NHL patient with dup(1)(q21q25) and rearrangement in the FCGR2B region has also been identified.
Abnormal Protein None
Oncogenesis Contribution of FCGR2B deregulation to lymphoma development or progression is currently not known.
  

Bibliography

The IgG Fc receptor, FcgammaRIIB, is a target for deregulation by chromosomal translocation in malignant lymphoma.
Callanan MB, Le Baccon P, Mossuz P, Duley S, Bastard C, Hamoudi R, Dyer MJ, Klobeck G, Rimokh R, Sotto JJ, Leroux D
Proceedings of the National Academy of Sciences of the United States of America. 2000 ; 97 (1) : 309-314.
PMID 10618414
 
Deregulation of FCGR2B expression by 1q21 rearrangements in follicular lymphomas.
Chen W, Palanisamy N, Schmidt H, Teruya-Feldstein J, Jhanwar SC, Zelenetz AD, Houldsworth J, Chaganti RS
Oncogene. 2001 ; 20 (52) : 7686-7693.
PMID 11753646
 
Fc receptor biology.
Daë M
Annual review of immunology. 1997 ; 15 : 203-234.
PMID 9143687
 
Immune inhibitory receptors.
Ravetch JV, Lanier LL
Science (New York, N.Y.). 2000 ; 290 (5489) : 84-89.
PMID 11021804
 

Citation

This paper should be referenced as such :
Callanan, M ; Leroux, D
FCGR2B (Fc fragment of IgG, low affinity IIb, receptor (CD32))
Atlas Genet Cytogenet Oncol Haematol. 2002;6(2):113-114.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Genes/FCGR2BID397.html


Other Leukemias implicated (Data extracted from papers in the Atlas) [ 7 ]
  t(1;14)(q21;q32) FCGR2B/IGH
t(1;14)(q21;q32) BCL9/IGH::t(1;22)(q21;q11) IGL/BCL9
t(1;14)(q21;q32) FCGR2B/IGH
t(1;14)(q21;q32) FCRL4/IGH
t(1;14)(q21;q32)::t(1;22)(q21;q11)
t(1;14)(q21;q32) MUC1/IGH
t(8;9)(q24;q13)


External links

Nomenclature
Cards
AtlasFCGR2BID397.txt
Aliases
Genomic and cartography
Gene and transcription
RefSeq transcript (Entrez)
RefSeq genomic (Entrez)
SOURCE (Princeton)Expression in : [Datasets]   [Normal Tissue Atlas]  [carcinoma Classsification]  [NCI60]
BioGPS (Tissue expression)2213
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 Oct 18 17:36:19 CEST 2018

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