Transcriptomics

Dataset Information

34

A novel unusual chromosome 11 abnormality: a homozygous somatic deletion of the entire WT1 gene within a heterozygous 11p13 deletion and UPD limited to 11p15 in a Wilms tumor and establishment of an immortalized cell line


ABSTRACT: We describe a stromal predominant Wilms tumor with a complex, tumor specific chromosome 11 aberration: a homozygous deletion of the entire WT1 gene within a heterozygous 11p13 deletion and an additional region of uniparental disomy (UPD) limited to 11p15.5-p15.2 including the IGF2 gene. The tumor carried a heterozygous p.T41A mutation in CTNNB1. Cells established from the tumor carried the same chromosome 11 aberration, but a different, homozygous p.S45Δ CTNNB1 mutation. Uniparental disomy (UPD) 3p21.3pter lead to the homozygous CTNNB1 mutation. The tumor cell line was immortalized with telomerase (TERT) and a novel triple ts mutant SV40 large T antigen (LT). This cell line is cytogenetically stable and can be grown indefinitely. It is a valuable tool to study the effect of a complete lack of WT1 in tumor cells. The tumor cell line and the immortalized cells have a limited potential for muscle/osteogenic/adipogenic differentiation similar to all other WT1 mutant cell lines. Array expression experiment of Wilms tumor cell lines

ORGANISM(S): Homo sapiens  

SUBMITTER: Jaume Mora  Brigitte Royer-Pokora   Manfred Beier   Barbara Leube   Katharina Löhers   Nunu Arora   Parmjit Jat   Carmen Torres   Artur Brandt    

PROVIDER: E-GEOD-71265 | ArrayExpress| 2016-06-04

SECONDARY ACCESSION(S): GSE71265PRJNA290728

REPOSITORIES: GEO, ArrayExpress

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Publications

Establishment of a Conditionally Immortalized Wilms Tumor Cell Line with a Homozygous WT1 Deletion within a Heterozygous 11p13 Deletion and UPD Limited to 11p15.

Brandt Artur A   Löhers Katharina K   Beier Manfred M   Leube Barbara B   de Torres Carmen C   Mora Jaume J   Arora Parineeta P   Jat Parmjit S PS   Royer-Pokora Brigitte B  

PloS one 20160523 5


We describe a stromal predominant Wilms tumor with focal anaplasia and a complex, tumor specific chromosome 11 aberration: a homozygous deletion of the entire WT1 gene within a heterozygous 11p13 deletion and an additional region of uniparental disomy (UPD) limited to 11p15.5-p15.2 including the IGF2 gene. The tumor carried a heterozygous p.T41A mutation in CTNNB1. Cells established from the tumor carried the same chromosome 11 aberration, but a different, homozygous p.S45Δ CTNNB1 mutation. Unip  ...[more]

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