TFB2M


Description

The TFB2M (transcription factor B2, mitochondrial) is a protein-coding gene located on chromosome 1.

TFB2M, also known as Dimethyladenosine transferase 2, mitochondrial, is an enzyme encoded by the TFB2M gene. It acts as a transcription initiation factor for the mitochondrial RNA polymerase (POLRMT). Its paralog, TFB1M, can perform a similar function, but only in vitro.

TFB2M is an S-adenosyl-L-methionine-dependent rRNA methyltransferase that may methylate two specific adjacent adenosines in the loop of a conserved hairpin near the 3'-end of 12S mitochondrial rRNA. It is a component of the mitochondrial transcription initiation complex, composed of TFB2M, TFAM, and POLRMT, which is essential for basal transcription of mitochondrial DNA. Within this complex, TFAM recruits POLRMT to a specific promoter, while TFB2M induces structural changes in POLRMT to enable promoter opening and trapping of the DNA non-template strand. TFB2M stimulates transcription independently of its methyltransferase activity.

TFB2M is also known as Hkp1, h-mtTFB, h-mtTFB2, hTFB2M, mtTFB2.

Associated Diseases



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