FEATURES

FAMILY

DESCRIPTION

Also known as RRP8_HUMAN, RRP8, KIAA0409, NML. Essential component of the eNoSC (energy-dependent nucleolar silencing) complex, a complex that mediates silencing of rDNA in response to intracellular energy status and acts by recruiting histone-modifying enzymes. The eNoSC complex is able to sense the energy status of cell: upon glucose starvation, elevation of NAD(+)/NADP(+) ratio activates SIRT1, leading to histone H3 deacetylation followed by dimethylation of H3 at 'Lys-9' (H3K9me2) by SUV39H1 and the formation of silent chromatin in the rDNA locus. In the complex, RRP8 binds to H3K9me2 and probably acts as a methyltransferase. Its substrates are however unknown. Component of the eNoSC complex, composed of SIRT1, SUV39H1 and RRP8.

EXTERNAL LINKS

RRP8

Mutations

Mutation(s) distribution

Mutations table