ERCC6 (CSB) and EHMT2 (G9a) positively regulate rRNA expression

Stable Identifier
R-HSA-427389
Type
Pathway
Species
Homo sapiens
Compartment
Synonyms
Activation of rRNA Expression by ERCC6 (CSB) and EHMT2 (G9a), Activation of rRNA Expression by Cockayne Syndrome Protein CSB and Histone Methyltransferase G9a
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About half of the rRNA genes in the genome are actively expressed, being transcribed by RNA polymerase I (reviewed in Nemeth and Langst 2008, Bartova et al. 2010, Goodfellow and Zomerdijk 2012, Grummt and Langst 2013). As inferred from mouse, those genes that are expressed are activated by ERCC6 (also known as Cockayne Syndrome protein, CSB) which interacts with TTF-I bound to the T0 terminator region (also know as the Sal Box) of rRNA genes (Yuan et al. 2007, reviewed in Birch and Zomerdijk 2008, Grummt and Langst 2013). ERCC6 recruits the histone methyltransferase EHMT2 (also known as G9a) which dimethylates histone H3 at lysine-9 in the coding region of rRNA genes. The dimethylated lysine is bound by CBX3 (also known as Heterochromatic Protein-1gamma, HP1gamma) and increases expression of the rRNA gene. Continuing dimethylation depends on continuing transcription. Mutations in CSB result in dysregulation of RNA polymerase I transcription, which plays a role in the symptoms of Cockayne Syndrome (reviewed in Hannan et al. 2013).

Literature References
PubMed ID Title Journal Year
17707230 Activation of RNA polymerase I transcription by cockayne syndrome group B protein and histone methyltransferase G9a

Yuan, X, Feng, W, Imhof, A, Grummt, I, Zhou, Y

Mol Cell 2007
20026667 Structure and epigenetics of nucleoli in comparison with non-nucleolar compartments

Bártová, E, Horáková, AH, Uhlírová, R, Raska, I, Galiová, G, Orlova, D, Kozubek, S

J. Histochem. Cytochem. 2010
23150253 Basic mechanisms in RNA polymerase I transcription of the ribosomal RNA genes

Goodfellow, SJ, Zomerdijk, JC

Subcell. Biochem. 2012
23153826 Dysregulation of RNA polymerase I transcription during disease

Hannan, KM, Sanij, E, Rothblum, LI, Hannan, RD, Pearson, RB

Biochim. Biophys. Acta 2013
23063748 Epigenetic control of RNA polymerase I transcription in mammalian cells

Grummt, I, Längst, G

Biochim. Biophys. Acta 2013
18948749 Chromatin organization of active ribosomal RNA genes

Németh, A, Längst, G

Epigenetics 2008
18631128 Structure and function of ribosomal RNA gene chromatin

Birch, JL, Zomerdijk, JC

Biochem Soc Trans 2008
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