DNA-directed RNA polymerase, mitochondrial

Definition:

DNA-dependent RNA polymerase catalyzes the transcription of mitochondrial DNA into RNA using the four ribonucleoside triphosphates as substrates. Component of the mitochondrial transcription initiation complex, composed at least of TFB2M, TFAM and POLRMT that is required for basal transcription of mitochondrial DNA. In this complex, TFAM recruits POLRMT to a specific promoter whereas TFB2M induces structural changes in POLRMT to enable promoter opening and trapping of the DNA non-template strand. Has DNA primase activity. Catalyzes the synthesis of short RNA primers that are necessary for the initiation of lagging-strand DNA synthesis from the origin of light-strand DNA replication (OriL).

References:

[1]. Hauke S Hillen, et al. Structural Basis of Mitochondrial Transcription Initiation. Cell. 2017 Nov 16;171(5):1072-1081.e10. [Content Brief]

[2]. Michal Minczuk, et al. TEFM (c17orf42) is necessary for transcription of human mtDNA. Nucleic Acids Res. 2011 May;39(10):4284-99. [Content Brief]

[3]. Monika Oláhová, et al. POLRMT mutations impair mitochondrial transcription causing neurological disease. Nat Commun. 2021 Feb 18;12(1):1135. [Content Brief]

[4]. Sjoerd Wanrooij, et al. Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro. Proc Natl Acad Sci U S A. 2008 Aug 12;105(32):11122-7. [Content Brief]

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