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)

Function: 1) S-adenosyl-L-methionine-dependent methyltransferase 2) specifically dimethylates mitochondrial 12S rRNA at the conserved stem loop 3) required for basal transcription of mitochondrial DNA, probably via its interaction with POLRMT & TFAM - stimulates transcription independent of methyltransferase activity 4) interacts with mitochondrial RNA polymerase POLRMT 5) interacts with TFAM Structure: - Belongs to the rRNA adenine N(6)-methyltransferase family, KsgA subfamily Compartment: mitochondrion Expression: - ubiquitously expressed - induced by nuclear respiratory factors NRF1 & NRF2/GABPB2 & PGC-1 coactivators Pathology: - variations in TFB1M may influence aminoglycoside-induced deafness

General

methyltransferase mitochondrial protein

Properties

SIZE: MW = 40 kD entity length = 346 aa COMPARTMENT: mitochondria MOTIF: active site

Database Correlations

OMIM correlations UniProt Q8WVM0 Pfam PF00398

References

UniProt :accession Q8WVM0