RRNA small subunit pseudouridine methyltransferase Nep1

RRNA small subunit pseudouridine methyltransferase Nep1 (, Nep1, nucleolar essential protein 1) is an enzyme with systematic name S-adenosyl-L-methionine:18S rRNA (pseudouridine1191-N1)-methyltransferase. This enzyme catalyses the following chemical reaction


 * S-adenosyl-L-methionine + pseudouridine1191 in yeast 18S rRNA $$\rightleftharpoons$$ S-adenosyl-L-homocysteine + N1-methylpseudouridine1191 in yeast 18S rRNA

This enzyme recognizes specific pseudouridine residues (Psi) in small subunits of ribosomal RNA based on the local RNA structure.

A point mutation in the ribosome biogenesis factor Nep1 impairs its nucleolar localisation and RNA binding and causes the Bowen-Conradi syndrome. .