EZH2

By Jennifer Sokolowski, MD, PhD.

By Christina Towers, PhD.

EZH1 is part of the Polycomb-group family of proteins, which are responsible for remodeling chromatin in genes and modulating epigenetic silencing during development.  Specifically, EZHI is a component of PRC2, or polycomb repressive complex-2.  PRC2 interacts and modifies the histone “H3”, and is critical in maintaining gene repression.

Over the years muscular dystrophies have become a popular area of research. These are a group of inherited disorders that involve an increase in muscle weakness over time. These disorders greatly decrease the quality of life and there are no known cures. Research in this area appears to have excelled in the past two years with findings related to the genes NCoR and EZH2.

The jumonji (JMJ) gene, obtained by a gene trap strategy, is essential for embryogenesis and is suggested to play important roles in cell growth during development. The amino acid sequence of the JMJ protein includes a nuclear localization signal and a DNA binding motif called the AT-rich interactive domain (ARID). Unlike the other members of the JARID family of proteins, JARID2 has a long N-terminal moiety devoid of characterized domains.

Enhancer of Zeste homolog 2 (EZH2) is the methyltransferase enzyme responsible for trimethylating lysine 27 on histone H3 to produce H3K27Me3. EZH2 is a polycomb group protein that is an essential epigenetic regulator that is often found deregulated in a wide variety of malignant cancer types.