TY  - JOUR
AU  - Jeibmann, Astrid
AU  - Eikmeier, Kristin
AU  - Linge, Anna
AU  - Kool, Marcel
AU  - Koos, Björn
AU  - Schulz, Jacqueline
AU  - Albrecht, Stefanie
AU  - Bartelheim, Kerstin
AU  - Frühwald, Michael C
AU  - Pfister, Stefan
AU  - Paulus, Werner
AU  - Hasselblatt, Martin
TI  - Identification of genes involved in the biology of atypical teratoid/rhabdoid tumours using Drosophila melanogaster.
JO  - Nature Communications
VL  - 5
SN  - 2041-1723
CY  - London
PB  - Nature Publishing Group
M1  - DKFZ-2017-02180
SP  - 4005
PY  - 2014
AB  - Atypical teratoid/rhabdoid tumours (AT/RT) are malignant brain tumours. Unlike most other human brain tumours, AT/RT are characterized by inactivation of one single gene, SMARCB1. SMARCB1 is a member of the evolutionarily conserved SWI/SNF chromatin remodelling complex, which has an important role in the control of cell differentiation and proliferation. Little is known, however, about the pathways involved in the oncogenic effects of SMARCB1 inactivation, which might also represent targets for treatment. Here we report a comprehensive genetic screen in the fruit fly that revealed several genes not yet associated with loss of snr1, the Drosophila homologue of SMARCB1. We confirm the functional role of identified genes (including merlin, kibra and expanded, known to regulate hippo signalling pathway activity) in human rhabdoid tumour cell lines and AT/RT tumour samples. These results demonstrate that fly models can be employed for the identification of clinically relevant pathways in human cancer.
KW  - Chromosomal Proteins, Non-Histone (NLM Chemicals)
KW  - DNA-Binding Proteins (NLM Chemicals)
KW  - Drosophila Proteins (NLM Chemicals)
KW  - Intracellular Signaling Peptides and Proteins (NLM Chemicals)
KW  - Membrane Proteins (NLM Chemicals)
KW  - Neurofibromin 2 (NLM Chemicals)
KW  - SMARCB1 Protein (NLM Chemicals)
KW  - SMARCB1 protein, human (NLM Chemicals)
KW  - Snr1 protein, Drosophila (NLM Chemicals)
KW  - Transcription Factors (NLM Chemicals)
KW  - Tumor Suppressor Proteins (NLM Chemicals)
KW  - expanded protein, Drosophila (NLM Chemicals)
KW  - kibra protein, Drosophila (NLM Chemicals)
KW  - merlin, Drosophila (NLM Chemicals)
KW  - Protein-Serine-Threonine Kinases (NLM Chemicals)
KW  - hpo protein, Drosophila (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:24892285
DO  - DOI:10.1038/ncomms5005
UR  - https://inrepo02.dkfz.de/record/126065
ER  -