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

2015-02-01   Jean-Loup Huret 

1.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.

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

Genes Involved and Proteins

Gene name
CCND1 (B-cell leukemia/lymphoma 1)
Location
11q13.3
Protein description
Binds and activates the G1 cyclin dependent kinases; Phosphorylates SMAD3 and inhibits its transcriptional activity and antiproliferative function.
Gene name
FSTL3 (follistatin-like 3 (secreted glycoprotein))
Location
19p13.3
Protein description
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

Description

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

Bibliography

Pubmed IDLast YearTitleAuthors
96714161998FLRG (follistatin-related gene), a new target of chromosomal rearrangement in malignant blood disorders.Hayette S et al
84996401993Rearrangement and overexpression of the BCL-1/PRAD-1 gene in intermediate lymphocytic lymphomas and in t(11q13)-bearing leukemias.Rimokh R et al

Summary

Fusion gene

FSTL3/CCND1 FSTL3 (19p13.3) CCND1 (11q13.3) M|FSTL3/CCND1 FSTL3 (19p13.3) CCND1 (11q13.3) M t(11;19)(q13;p13)

Citation

Jean-Loup Huret

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

Atlas Genet Cytogenet Oncol Haematol. 2015-02-01

Online version: http://atlasgeneticsoncology.org/haematological/1527/t(11;19)(q13;p13)-fstl3-ccnd1

External Links