The DOI matches its Crossref record; 30 of the 30 references it cites were found in a registry or an index.
Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA
Abstract
Characterization of the basic transcription machinery of mammalian mitochondrial DNA (mtDNA) is of fundamental biological interest and may also lead to therapeutic interventions for human diseases associated with mitochondrial dysfunction. Here we report that mitochondrial transcription factors B1 (TFB1M) and B2 (TFB2M) are necessary for basal transcription of mammalian mitochondrial DNA (mtDNA). Human TFB1M and TFB2M are expressed ubiquitously and can each support promoter-specific mtDNA transcription in a pure recombinant in vitro system containing mitochondrial RNA polymerase (POLRMT) and mitochondrial transcription factor A. Both TFB1M and TFB2M interact directly with POLRMT, but TFB2M is at least one order of magnitude more active in promoting transcription than TFB1M. Both factors are highly homologous to bacterial rRNA dimethyltransferases, which suggests that an RNA-modifying enzyme has been recruited during evolution to function as a mitochondrial transcription factor. The presence of two proteins that interact with mammalian POLRMT may allow flexible regulation of mtDNA gene expression in response to the complex physiological demands of mammalian metabolism.
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“Tfam is ubiquitously expressed and absolutely required for mtDNA transcription (8,13).”
From Increased mitochondrial mass in mitochondrial myopathy mice (2002) · cites this paper as [13]
Citations
Cites
Sequence and organization of the human mitochondrial genome
Anderson, S., Bankier, A. T., Barrell, B. G., de Bruijn, M. H. L., Coulson, A. R., Drouin, J., Eperon, I. C., Nierlich, D. P., Roe, B. A., Sanger, F., et al. (1981) · Nature · doi:10.1038/290457a0 · Cited by 9,740
Mitochondrial diseases in man and mouse
Wallace, D. C. (1999) · Science · doi:10.1126/science.283.5407.1482 · Cited by 3,060
tRNA punctuation model of RNA processing in human mitochondria
Ojala, D., Montoya, J., & Attardi, G. M. (1981) · Nature · doi:10.1038/290470a0 · Cited by 2,561
Sequence and gene organization of mouse mitochondrial DNA.
Bibb, M., Etten, R. V., Wright, C. T., Walberg, M. W., & Clayton, D. A. (1981) · Cell · doi:10.1016/0092-8674(81)90300-7 · Cited by 1,619
Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice
Larsson, N.-G., Wang, J., Wilhelmsson, H., Oldfors, A., Rustin, P., Lewandoski, M., Barsh, G. S., & Clayton, D. A. (1998) · Nature Genetics · doi:10.1038/ng0398-231 · Cited by 1,594
Self-citationThe genetics and pathology of oxidative phosphorylation
Smeitink, J. A. M., van den Heuvel, L., & DiMauro, S. (2001) · Nature Reviews Genetics · doi:10.1038/35072063 · Cited by 733
Replication And Transcription Of Vertebrate Mitochondrial Dna
Clayton, D. A. (1991) · Annual Review of Cell Biology · doi:10.1146/annurev.cb.07.110191.002321 · Cited by 652
Similarity of Human Mitochondrial Transcription Factor 1 to High Mobility Group Proteins
Parisi, M. A., & Clayton, D. A. (1991) · Science · doi:10.1126/science.2035027 · Cited by 548
MOLECULAR GENETIC ASPECTS OF HUMAN MITOCHONDRIAL DISORDERS
Larsson, N.-G., & Clayton, D. A. (1995) · Annual Review of Genetics · doi:10.1146/annurev.ge.29.120195.001055 · Cited by 493
Self-citationTRANSCRIPTION OF THE MAMMALIAN MITOCHONDRIAL GENOME
Clayton, D. A. (1984) · Annual Review of Biochemistry · doi:10.1146/annurev.bi.53.070184.003041 · Cited by 446
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Mitochondrial DNA mutations in human disease
Taylor, R. W., & Turnbull, D. M. (2005) · Nature Reviews Genetics · doi:10.1038/nrg1606 · Cited by 1,922
Transcriptional paradigms in mammalian mitochondrial biogenesis and function
Scarpulla, R. C. (2008) · Physiological Reviews · doi:10.1152/physrev.00025.2007 · Cited by 1,666
“Two isoforms of h-mtTFB, termed TFB1M and TFB2M, have been identified independently (65).”
BackgroundInfluentialTranscriptional regulatory circuits controlling mitochondrial biogenesis and function
Kelly, D. P., & Scarpulla, R. C. (2004) · Genes & Development · doi:10.1101/gad.1177604 · Cited by 1,243
“These include genes for Tfam (Larsson et al. 1998; Rantanen et al. 2001) and the newly discovered TFB factors (Falkenberg et al. 2002; McCulloch et al. 2002) involved in mitochondrial transcription and DNA replication (Rantanen et al. 2001).”
BackgroundTranscriptional control of mitochondrial biogenesis: the central role of PGC-1
Ventura-Clapier, R., Garnier, A., & Veksler, V. (2008) · Cardiovascular Research · doi:10.1093/cvr/cvn098 · Cited by 932
“Additionally, two proteins that interact with the mammalian mitochondrial RNA polymerase and Tfam, TFB1M and TFB2M, can support promoter-specific mtDNA transcription.(15) Although usually complex and promoter-specific, certain DNA binding motifs are present in the promoters of several of genes encoding oxidative phosp…”
BackgroundMitochondrial transcription factor A regulates mtDNA copy number in mammals
Ekstrand, M. I. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh109 · Cited by 919
Metadata
- Authors
- Falkenberg, M., Gaspari, M., Rantanen, A., Trifunovic, A., Larsson, N.-G., & Gustafsson, C. M.
- Affiliations
- Karolinska Institutet (SE)
- Corresponding author
- Larsson, Nils-Göran
- Gustafsson, Claes M.
- Venue
- Nature Genetics
- Published
- 2002-06-17
- Volume
- 31
- Issue
- 3
- Pages
- 289-294
- ISSN
- 1061-4036 (ISSN-L)
- 1546-1718
- OpenAlex
- W2081360068
- Cited by
- 598
- References
- 30
- Citations by year
- 2026: 5 · 2025: 14 · 2024: 14 · 2023: 14 · 2022: 15 · 2021: 18 · 2020: 19 · 2019: 24 · 2018: 23 · 2017: 31 · 2016: 17 · 2015: 26 · 2014: 22 · 2013: 30 · 2012: 30
- FWCI
- 10.08
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Genetics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Genomics and Chromatin Dynamics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Keywords
- mitochondrial transcription factors, TFB1M, TFB2M, mitochondrial transcription, mitochondrial RNA polymerase, transcriptional regulation, mitochondrial dysfunction
- MeSH terms
- Amino Acid Sequence, Animals, DNA-Binding Proteins, DNA, Mitochondrial, Humans, Leukocytes, Methyltransferases, Molecular Sequence Data, Promoter Regions, Genetic, Recombinant Proteins, DNA-Directed RNA Polymerases, Transcription Factors, Transcription, Genetic, Sequence Homology, Amino Acid, Mitochondrial Proteins, Mice, In Vitro Techniques
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