WBSCR22:TRMT112 methylates guanosine-1639 of 18S rRNA yielding 7-methylguanosine-1639

Stable Identifier
Reaction [transition]
Homo sapiens
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The WBSCR22:TRMT112 complex, homolog of the Bud23:Trm112 complex in yeast (Ounap et al. 2013), methylates guanosine-1639 of 18S rRNA at the N(7) position of the guanine base (Haag et al. 2015, Zorbas et al. 2015). The WBSCR22:TRMT112 complex but not its methylase activity is required for efficient processing of precursor rRNA at site 2 and site 3 (Haag et al. 2015, Zorbas et al. 2015). Hemizygosity at the region containing WBSCR22 causes Williams-Beuren syndrome (Doll and Grzeschik 2001).

Literature References
PubMed ID Title Journal Year
25851604 The human 18S rRNA base methyltransferases DIMT1L and WBSCR22-TRMT112 but not rRNA modification are required for ribosome biogenesis

Nicolas, E, Huvelle, E, Zorbas, C, Heurgué-Hamard, V, Wacheul, L, Lafontaine, DL

Mol. Biol. Cell 2015
24086612 The human WBSCR22 protein is involved in the biogenesis of the 40S ribosomal subunits in mammalian cells

Kurg, A, Kurg, R, Õunap, K, Käsper, L

PLoS ONE 2013
25525153 WBSCR22/Merm1 is required for late nuclear pre-ribosomal RNA processing and mediates N7-methylation of G1639 in human 18S rRNA

Haag, S, Kretschmer, J, Bohnsack, MT

RNA 2015
11978965 Characterization of two novel genes, WBSCR20 and WBSCR22, deleted in Williams-Beuren syndrome

Grzeschik, KH, Doll, A

Cytogenet. Cell Genet. 2001
Event Information
Go Biological Process
Catalyst Activity

rRNA (guanine-N7-)-methyltransferase activity of WBSCR22:TRIMT112 [nucleoplasm]

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