147040

KCTD11

C17orf36|KCASH1|REN|REN/KCTD11;potassium channel tetramerisation domain containing 11;KCTD11;potassium channel tetramerisation domain containing 11

BTB/POZ domain-containing protein KCTD11|potassium channel tetramerization domain containing 11|retinoic acid, EGF, NGF induced gene protein|retinoic acid, EGF, NGF induced gene/potassium channel tetramerization domain containing 11

17p13.1

protein-coding

Medulloblastoma (MB) is the most common malignant brain tumor of childhood, and the most frequent associated genetic alteration is loss of heterozygosity on chromosome region 7p13. Two genes mapping to this region, KCTD11 (alias REN) andHIC1, have been proposed as involved in MB pathogenesis. We used real-time polymerase chain reaction in 20 tissue samples of primary MB to examine the transcriptional level of the two genes, with reference to two types of controls:adult cerebellum and fetal neural stem cells. A significant reduction of KCTD11 expression relative to adult normal cerebellum was detected in 14 of 20 (70%) ofMB samples. Neural stem cells had even lower levels of KCTD11 expression than did MB. HIC1 gene expression was low ( approximately 100 times lower than KCTD11 expression) in MB, and low also in both adult cerebellum and neural stem cells. Hypermethylation of the 5'UTR or the central region of HIC1 (or both) was detected in a significant number of MB samples, as well as in cerebellum and neural stem cells. Our data suggest that KCTD11 may play an important role in MBtumorigenesis, but do not support the role of HIC1 in this tumor development. Weargue that recognition of the gene or genes important in MB tumorigenesis depends in part on defining an appropriate control.