Epigenomic Alterations Define Lethal CIMP positive Ependymomas of Infancy

ABSTRACT: Ependymomas are common childhood brain tumors that occur throughout the nervous system, but are most common in the pediatric hindbrain. Current standard therapy comprises surgery and radiation, but not cytotoxic chemotherapy as it does not further increase survival. Whole-genome and whole-exome sequencing of 47 hindbrain ependymomas reveals an extremely low mutation rate, and zero significant recurrent somatic SNVs. While devoid of recurrent SNVs and focal copy number aberrations, poor prognosis hindbrain ependymomas exhibit a CpG island methylator phenotype (CIMP). Transcriptional silencing driven by CpG methylation converges exclusively on targets of the polycomb repressor complex 2 (PRC2) that represses expression of differentiation genes through tri-methylation of H3K27. CIMP-positi

ORGANISM(S): Homo sapiens

SUBMITTER: Stephen Christopher 

PROVIDER: E-GEOD-55920 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress