2006
DOI: 10.1016/j.molcel.2006.11.024
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Initiation and Beyond: Multiple Functions of the Human Mitochondrial Transcription Machinery

Abstract: Mitochondria contain their own DNA (mtDNA) that is expressed and replicated by nucleus-encoded factors imported into the organelle. Recently, the core human mitochondrial transcription machinery has been defined, comprising a bacteriophage-related mtRNA polymerase (POLRMT), an HMG-box transcription factor (h-mtTFA), and two transcription factors (h-mtTFB1 and h-mtTFB2) that also serve as rRNA methyltransferases. Here, we describe these transcription components as well as recent insights into the mechanism of h… Show more

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Cited by 313 publications
(327 citation statements)
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References 98 publications
(138 reference statements)
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“…Meanwhile, the ancestral homologue of h-mtTFA/Abf2p was initially important for mtDNA packaging, but at some point in metazoan evolution, mtTFA acquired the ability to stimulate transcription through the acquisition of a C-terminal tail (43) and coevolution of specific binding sites in mtDNA adjacent to promoters. This also likely allowed h-mtTFA to acquire a more exquisite ability to regulate the multifunctional LSP, which has a role in priming mtDNA replication as well as transcription (6)(7)(8).…”
Section: Discussionmentioning
confidence: 99%
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“…Meanwhile, the ancestral homologue of h-mtTFA/Abf2p was initially important for mtDNA packaging, but at some point in metazoan evolution, mtTFA acquired the ability to stimulate transcription through the acquisition of a C-terminal tail (43) and coevolution of specific binding sites in mtDNA adjacent to promoters. This also likely allowed h-mtTFA to acquire a more exquisite ability to regulate the multifunctional LSP, which has a role in priming mtDNA replication as well as transcription (6)(7)(8).…”
Section: Discussionmentioning
confidence: 99%
“…The two rRNAs assemble with nucleus-encoded mitochondrial ribosomal proteins to generate mitochondrial ribosomes. Finally, transcripts from the LSP are also used as primers for mtDNA replication (5,6), thus LSP transcription serves a dual role in gene expression and mtDNA maintenance (7,8).…”
mentioning
confidence: 99%
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“…Each mitochondrion contains 4-6 copies of the double-stranded circular 16 kilobase long mitochondrial genome. MtDNA needs to be tightly preserved because more than 93% of the mitochondrial genome has to be accurately transcribed into 13 individual messenger RNAs coding for the essential mitochondrial proteins that are part of the mitochondrial electron transport chain (oxidative phosphorylation) (1). MtDNA also encodes 2 mitochondrialspecific ribosomal RNAs and 22 transfer RNAs that are essential for protein synthesis inside mitochondria.…”
mentioning
confidence: 99%
“…In mammals, mtDNA is maternally inherited and essential because it encodes genes required for oxidative phosphorylation. For example, human mtDNA is a 16,569 basepair, double-stranded, circular molecule harboring thirty-seven genes: thirteen mRNAs, two rRNAs, and twenty-two tRNAs (1). The remaining ∼1;500 proteins in mitochondria (2) are nuclear gene products that are imported into the organelle, including many required for expression and replication of mtDNA (3).…”
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confidence: 99%