AUTHOR=Aksoy Yasar Fatma Betul , Shingu Takashi , Zamler Daniel B. , Zaman Mohammad Fayyad , Chien Derek Lin , Zhang Qiang , Ren Jiangong , Hu Jian 

TITLE=Quaking but not parkin is the major tumor suppressor in 6q deleted region in glioblastoma

JOURNAL=Frontiers in Cell and Developmental Biology

VOLUME=10

YEAR=2022

URL=https://www.frontiersin.org/journals/cell-and-developmental-biology/articles/10.3389/fcell.2022.931387

DOI=10.3389/fcell.2022.931387

ISSN=2296-634X

ABSTRACT=<p>Glioblastoma (GBM) is a high-grade, aggressive brain tumor with dismal median survival time of 15 months. Chromosome 6q (Ch6q) is a hotspot of genomic alterations, which is commonly deleted or hyper-methylated in GBM. Two neighboring genes in this region, <italic>QKI</italic> and <italic>PRKN</italic> have been appointed as tumor suppressors in GBM. While a genetically modified mouse model (GEMM) of GBM has been successfully generated with <italic>Qk</italic> deletion in the central nervous system (CNS), <italic>in vivo</italic> genetic evidence supporting the tumor suppressor function of <italic>Prkn</italic> has not been established. In the present study, we generated a mouse model with <italic>Prkn</italic>-null allele and conditional <italic>Trp53</italic> and <italic>Pten</italic> deletions in the neural stem cells (NSCs) and compared the tumorigenicity of this model to our previous GBM model with <italic>Qk</italic> deletion within the same system. We find that <italic>Qk</italic> but not <italic>Prkn</italic> is the potent tumor suppressor in the frequently altered Ch6q region in GBM.</p>