TY  - JOUR
AU  - Wegert, Jenny
AU  - Ishaque, Naveed
AU  - Vardapour, Romina
AU  - Geörg, Christina
AU  - Gu, Zuguang
AU  - Bieg, Matthias
AU  - Ziegler, Barbara
AU  - Bausenwein, Sabrina
AU  - Nourkami, Nasenien
AU  - Ludwig, Nicole
AU  - Keller, Andreas
AU  - Grimm, Clemens
AU  - Kneitz, Susanne
AU  - Williams, Richard D
AU  - Chagtai, Tas
AU  - Pritchard-Jones, Kathy
AU  - van Sluis, Peter
AU  - Volckmann, Richard
AU  - Koster, Jan
AU  - Versteeg, Rogier
AU  - Acha, Tomas
AU  - O'Sullivan, Maureen J
AU  - Bode, Peter K
AU  - Niggli, Felix
AU  - Tytgat, Godelieve A
AU  - van Tinteren, Harm
AU  - van den Heuvel-Eibrink, Marry M
AU  - Meese, Eckart
AU  - Vokuhl, Christian
AU  - Leuschner, Ivo
AU  - Graf, Norbert
AU  - Eils, Roland
AU  - Pfister, Stefan
AU  - Kool, Marcel
AU  - Gessler, Manfred
TI  - Mutations in the SIX1/2 pathway and the DROSHA/DGCR8 miRNA microprocessor complex underlie high-risk blastemal type Wilms tumors.
JO  - Cancer cell
VL  - 27
IS  - 2
SN  - 1535-6108
CY  - Cambridge, Mass.
PB  - Cell Press
M1  - DKFZ-2017-03785
SP  - 298 - 311
PY  - 2015
AB  - Blastemal histology in chemotherapy-treated pediatric Wilms tumors (nephroblastoma) is associated with adverse prognosis. To uncover the underlying tumor biology and find therapeutic leads for this subgroup, we analyzed 58 blastemal type Wilms tumors by exome and transcriptome sequencing and validated our findings in a large replication cohort. Recurrent mutations included a hotspot mutation (Q177R) in the homeo-domain of SIX1 and SIX2 in tumors with high proliferative potential (18.1
KW  - DGCR8 protein, human (NLM Chemicals)
KW  - Homeodomain Proteins (NLM Chemicals)
KW  - MicroRNAs (NLM Chemicals)
KW  - Neoplasm Proteins (NLM Chemicals)
KW  - Nerve Tissue Proteins (NLM Chemicals)
KW  - RNA-Binding Proteins (NLM Chemicals)
KW  - SIX1 protein, human (NLM Chemicals)
KW  - SIX2 protein, human (NLM Chemicals)
KW  - DROSHA protein, human (NLM Chemicals)
KW  - Ribonuclease III (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:25670083
DO  - DOI:10.1016/j.ccell.2015.01.002
UR  - https://inrepo02.dkfz.de/record/127763
ER  -