mitochondrial dimethyladenosine transferase 1 (S-adenosylmethionine-6-N',N'-adenosyl(rRNA) dimethyltransferase 1, mitochondrial 12S rRNA dimethylase 1, mitochondrial transcription factor B1, mtTFB1, h-mtTFB1, h-mtTFB, TFB1M, hTFB1M)
Jump to navigation
Jump to search
Function
- S-adenosyl-L-methionine-dependent methyltransferase
- specifically dimethylates mitochondrial 12S rRNA at the conserved stem loop
- required for basal transcription of mitochondrial DNA, probably via its interaction with POLRMT & TFAM
- stimulates transcription independent of methyltransferase activity
- interacts with mitochondrial RNA polymerase POLRMT
- interacts with TFAM
Structure
- Belongs to the rRNA adenine N(6)-methyltransferase family, KsgA subfamily
Compartment
Expression
- ubiquitously expressed
- induced by nuclear respiratory factors NRF1 & NRF2/GABPB2 & PGC-1 coactivators
Pathology
- variations in TFB1M may influence aminoglycoside-induced deafness