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TGS1

Function

Catalyzes the 2 serial methylation steps for the conversion of the 7-monomethylguanosine (m(7)G) caps of snRNAs and snoRNAs to a 2,2,7-trimethylguanosine (m(2,2,7)G) cap structure. The enzyme is specific for guanine, and N7 methylation must precede N2 methylation. Hypermethylation of the m7G cap of U snRNAs leads to their concentration in nuclear foci, their colocalization with coilin and the formation of canonical Cajal bodies (CBs). Plays a role in transcriptional regulation.

Sequence Similarities

Belongs to the methyltransferase superfamily. Trimethylguanosine synthase family.

Tissue Specificity

Ubiquitously expressed. High expression in heart, skeletal muscle, kidney, liver and placenta.

Cellular localization

Alternative names

HCA137, NCOA6IP, PIMT, TGS1, Trimethylguanosine synthase, CLL-associated antigen KW-2, Cap-specific guanine-N(2) methyltransferase, Hepatocellular carcinoma-associated antigen 137, Nuclear receptor coactivator 6-interacting protein, PRIP-interacting protein with methyltransferase motif, PIPMT

swissprot:Q96RS0 entrezGene:96764 omim:606461