Histone H3 Mutations: An Updated View of Their Role in Chromatin Deregulation and Cancer.

ABSTRACT: In this review, we describe the attributes of histone H3 mutants identified in cancer. H3 mutants were first identified in genes encoding H3.3, in pediatric high-grade glioma, and subsequently in chondrosarcomas and giant cell tumors of bone (GCTB) in adolescents. The most heavily studied are the lysine to methionine mutants K27M and K36M, which perturb the target site for specific lysine methyltransferases and dominantly perturb methylation of corresponding lysines in other histone H3 proteins. We discuss recent progress in defining the consequences of these mutations on chromatin, including a newly emerging view of the central importance of the disruption of H3K36 modification in many distinct K to M histone mutant cancers. We also review new work exploring the role of H3.3 G34 mutants i

SUBMITTER: Lowe BR 

PROVIDER: S-EPMC6562757 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature