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t(11;19)(q13;p13) FSTL3/CCND1

Written2015-02Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Abstract Review on t(11;19)(q13;p13) FSTL3/CCND1, with data on clinics, and the genes involved.

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 1527

Clinics and Pathology

Disease B-cell chronic lymphocytic leukemia (B-CLL)
Epidemiology Only one case to date, a 67 year-old female patient (Rimokh et al., 1993; Hayette et al., 1998).
Prognosis No data

Cytogenetics

Cytogenetics Morphological Complex karyotype.

Genes involved and Proteins

Gene Name CCND1
Location 11q13.3
Protein Binds and activates the G1 cyclin dependent kinases; Phosphorylates SMAD3 and inhibits its transcriptional activity and antiproliferative function.
Gene Name FSTL3
Location 19p13.3
Protein Binds activin A and with lower affinity several other members of the TGF beta family; interacts with ADAM12, FN1 (fibronectin type 1,), and MLLT10 (Grusch, 2012).

Result of the chromosomal anomaly

Hybrid gene
Description The breaks occurred at nucleotide 2276 in the 3' untranslated region of the last CCND1 exon, and about 7 kbp upstream of FSTL3.
  

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

FSTL3 (follistatin-like 3 (secreted glycoprotein))
Grusch, M
Atlas Genet Cytogenet Oncol Haematol. 2013;17(3):151-154.
 
FLRG (follistatin-related gene), a new target of chromosomal rearrangement in malignant blood disorders.
Hayette S, Gadoux M, Martel S, Bertrand S, Tigaud I, Magaud JP, Rimokh R.
Oncogene. 1998 Jun 4;16(22):2949-54.
PMID 9671416
 
Rearrangement and overexpression of the BCL-1/PRAD-1 gene in intermediate lymphocytic lymphomas and in t(11q13)-bearing leukemias.
Rimokh R, Berger F, Delsol G, Charrin C, Berthéas MF, Ffrench M, Garoscio M, Felman P, Coiffier B, Bryon PA, et al.
Blood. 1993 Jun 1;81(11):3063-7.
PMID 8499640
 

Citation

This paper should be referenced as such :
Huret JL
t(11;19)(q13;p13) FSTL3/CCND1;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/t1119q13p13FSTL3-CCND1ID1527.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes CCND1 FSTL3

Translocations implicated (Data extracted from papers in the Atlas)

 t(11;19)(q13;p13) FSTL3/CCND1

External links

arrayMap (UZH-SIB Zurich)FSTL3 (19p13.3) CCND1 (11q13.3)

FSTL3 (19p13.3) CCND1 (11q13.3)

Mitelman databaset(11;19)(q13;p13) [Case List]    t(11;19)(q13;p13) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseFSTL3/CCND1 [MCList]  FSTL3 (19p13.3) CCND1 (11q13.3)
Mitelman databaseFSTL3/CCND1 [MCList]  FSTL3 (19p13.3) CCND1 (11q13.3)
 
Disease databaset(11;19)(q13;p13) FSTL3/CCND1
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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