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TFB1M

Function

Mitochondrial methyltransferase which uses S-adenosyl methionine to dimethylate two highly conserved adjacent adenosine residues (A1583 and A1584) within the loop of helix 45 at the 3-prime end of 12S rRNA, thereby regulating the assembly or stability of the small subunit of the mitochondrial ribosome (PubMed:12496758, PubMed:25305075, PubMed:31251801). Also required for basal transcription of mitochondrial DNA, probably via its interaction with POLRMT and TFAM. Stimulates transcription independently of the methyltransferase activity (PubMed:11809803, PubMed:12068295, PubMed:12897151).

Sequence Similarities

Belongs to the class I-like SAM-binding methyltransferase superfamily. rRNA adenine N(6)-methyltransferase family. KsgA subfamily.

Tissue Specificity

Ubiquitously expressed.

Cellular localization

Alternative names

CGI-75, TFB1M, Mitochondrial 12S rRNA dimethylase 1, Mitochondrial transcription factor B1, h-mtTFB, h-mtTFB1, hTFB1M, mtTFB1

swissprot:Q8WVM0 omim:607033 entrezGene:51106