Dimethyladenosine transferase 1, mitochondrial (EC 2.1.1.-) (Mitochondrial 12S rRNA dimethylase 1) (Mitochondrial transcription factor B1) (h-mtTFB) (h-mtTFB1) (hTFB1M) (mtTFB1) (S-adenosylmethionine-6-N', N'-adenosyl(rRNA) dimethyltransferase 1)
1_MAASG 6_ KLSTC 11_ RLPPL 16_ PTIRE 21_ IIKLL 26_ RLQAA 31_ KQLSQ 36_ NFLLD 41_ LRLTD 46_ KIVRK 51_ AGNLT 56_ NAYVY 61_ EVGPG 66_ PGGIT 71_ RSILN 76_ ADVAE 81_ LLVVE 86_ KDTRF 91_ IPGLQ 96_ MLSDA 101_ APGKL 106_ RIVHG 111_ DVLTF 116_ KVEKA 121_ FSESL 126_ KRPWE 131_ DDPPN 136_ VHIIG 141_ NLPFS 146_ VSTPL 151_ IIKWL 156_ ENISC 161_ RDGPF 166_ VYGRT 171_ QMTLT 176_ FQKEV 181_ AERLA 186_ ANTGS 191_ KQRSR 196_ LSVMA 201_ QYLCN 206_ VRHIF 211_ TIPGQ 216_ AFVPK 221_ PEVDV 226_ GVVHF 231_ TPLIQ 236_ PKIEQ 241_ PFKLV 246_ EKVVQ 251_ NVFQF 256_ RRKYC 261_ HRGLR 266_ MLFPE 271_ AQRLE 276_ STGRL 281_ LELAD 286_ IDPTL 291_ RPRQL 296_ SISHF 301_ KSLCD 306_ VYRKM 311_ CDEDP 316_ QLFAY 321_ NFREE 326_ LKRRK 331_ SKNEE 336_ KEEDD 341_AENYR
1: 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)