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t(6;10)(q22;q24) NFKB2/ROS1

Written2019-09Tatiana Gindina
R.M. Gorbacheva Memorial Institute of Children Oncology, Hematology and Transplantation at Pavlov First Saint-Petersburg State Medical University, Saint-Petersburg, Russian Federation / e-mail: tatgindina@gmail.com

Abstract Review on t(6;10)(q22;q24), with data on the genes involved.

Keywords Chromosome 6 ; Chromosome 10 ; t(6;10)(q22;q24) 

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424
ICD-Morpho 9702/3 Peripheral T-cell lymphoma, NOS; Anaplastic large cell lymphoma, ALK negative
Atlas_Id 1720

Clinics and Pathology

Disease Anaplastic large cell lymphoma, ALK negative
Note Only one case to date (Crescenzo et al., 2015). No individual data, no clinical data.
Prognosis No data

Genes involved and Proteins

Gene NameNFKB2
Location 10q24.32
Protein The gene encodes a subunit of the transcription factor complex nuclear factor-kappa-B. NFKB2 is a rapidly acting primary transcription factor found in all cell types. It is involved in the cellular responses to stimuli such as cytokines and stress and plays a key role in regulating the immunological response to infections.
Gene NameROS1
Location 6q22.1
Protein This proto-oncogene belongs to the subfamily of tyrosine kinase insulin receptor genes and expresses in a variety of tumor cell lines. ROS1 is an integral membrane protein with tyrosine kinase activity, that may function as a growth or differentiation factor receptor.

Result of the chromosomal anomaly

Hybrid gene
Note 5' NFKB2 - 3' ROS1
Description Nucleotide sequence analyses revealed that NFKB2 (exon 1-13) is fused to the intracytoplasmic domain of ROS1 accompanied by loss of the NFKB2 ankyrin region, that is necessary for the translation of constitutively active NFKB-fusion proteins and its oncogenic role [Crescenzo et al., 2015].
  
Fusion Protein
 
Oncogenesis NFKB2/ROS1 is a chimeric transcription factor with oncogenic potential that leads to the constitutive activation of STAT3 in the absence of either JAK-STAT3 mutations. The ectopic expression of NFKB2/ROS1 is associated with the phosphorylation of JAK (JAK2 - JAK3) and STAT3. NFKB2/ROS1 fusion induces the expression of genes involved in membrane trafficking, replication, protein export, TNF signaling, T cell activation. This, in turn, may lead to a widespread derangement of several metabolic and signaling pathways, which ultimately concur to neoplastic transformation [Crescenzo et al., 2015, Tabbo et al, 2016].
  

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Convergent mutations and kinase fusions lead to oncogenic STAT3 activation in anaplastic large cell lymphoma.
Crescenzo R, Abate F, Lasorsa E, Tabbo F, Gaudiano M, et al.
Cancer Cell. 2015; 27(4):516-532
PMID 25873174
 
Oncogenic kinase fusions: an evolving arens with innovative clinical opportunities.
Tabbo F, Pizzi M, Kyriakides PW, Ruggeri B, Inghirami G.
Oncotarget. 2016; 7(18):25064-25086
PMID 26943776
 

Citation

This paper should be referenced as such :
Gindina T
t(6;10)(q22;q24) NFKB2/ROS1;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0610q22q24NFKB2_ROS1ID1720.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(6;10)(q22;q24) NFKB2/ROS1

External links

Mitelman databaset(6;10)(q22;q24) [Case List]    t(6;10)(q22;q24) [Transloc-MCList] NFKB2/ROS1 [Fusion-MCList]
arrayMap (UZH-SIB Zurich)Morph ( 9702/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Wed Nov 13 22:14:07 CET 2019


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