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The protein encoded by this gene is a dimethyltransferase that methylates the conserved stem loop of mitochondrial 12S rRNA. The encoded protein also is part of the basal mitochondrial transcription complex and is necessary for mitochondrial gene expression. The methylation and transcriptional activities of this protein are independent of one another. Variations in this gene may influence the severity of aminoglycoside-induced deafness.
CGI75; CGI-75; Dimethyladenosine transferase 1, mitochondrial; h-mtTFB; h-mtTFB1; homolog of yeast mitochondrial transcription factor B; hTFB1M; mitochondrial 12S rRNA dimethylase 1; mitochondrial dimethyladenosine transferase 1; Mitochondrial transcription factor B1; mtTFB; mtTFB1; OTTHUMP00000017473; S-adenosylmethionine-6-N', N'-adenosyl(rRNA) dimethyltransferase 1; TFB1M; transcription factor B1, mitochondrial
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