Cited by 598Mitochondrial Function and PathologyGenomics and Chromatin DynamicsRNA and protein synthesis mechanisms

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Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

Falkenberg, Maria, Gaspari, Martina, Rantanen, Anja, Trifunovic, Aleksandra, Larsson, Nils-Göran, Gustafsson, Claes M.·2002·Nature Genetics

DOI 10.1038/ng909

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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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“In this context, our study highlights the lower expression of TFB2M, a key factor in mitochondrial transcription [38], and ABECE1 which also plays a role in mitochondrial translation quality control [39].”
Expanding the Genetic and Phenotypic Spectrum of POLRMT ‐Related Mitochondrial Disease
Fassad, M. R., Valenzuela, S., Oláhová, M., Collier, J. J., Knowles, C. V. Y., Mavraki, E., Elbracht, M., Güzel, N., Herberhold, T., Kurth, I., et al. (2025) · Clinical Genetics · doi:10.1111/cge.70011 · Cited by 4
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“In mammals, sperm mitochondria can transcribe and translate proteins through specialized mitochondrial RNA polymerases (e.g., POLRMT) and transcription factors (e.g., TFAM and TFB2M) (Falkenberg et al. 2002), supporting sperm vitality and function.”
Drosophila nuclear factor DREF regulates the expression of the mitochondrial DNA helicase and mitochondrial transcription factor B2 but not the mitochondrial translation factor B1
Fernández‐Moreno, M. Á., Hernández, R., Adán, C., Roberti, M., Bruni, F., Polosa, P. L., Cantatore, P., Matsushima, Y., Kaguni, L. S., & Garesse, R. (2013) · Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms · doi:10.1016/j.bbagrm.2013.07.006 · Cited by 4
“Notably, although mtTFB1 was originally described as a mtDNA transcription factor in vitro [20], that function has not been confirmed in vivo [21,22].”
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Gene-Eden-VIR Decreased Physical and Mental Fatigue in a Post Marketing Clinical Study That Followed FDA Guidelines; Results Support Microcompetition Theory
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“These include genes for Tfam [17] [18] and TFB1M and TFB2M [19] [20], and three human succinate dehydrogenase (complex II) subunit genes [21]-[23].”
Background
Phylogenetic Reconstruction Shows Independent Evolutionary Origins of Mitochondrial Transcription Factors from an Ancient Family of RNA Methyltransferase Proteins
Harris, A., & Goldman, A. D. (2018) · Journal of Molecular Evolution · doi:10.1007/s00239-018-9842-z · Cited by 4
“, animals) are unique in having two RAMTase orthologs that localize to the mitochondria (Falkenberg et al. 2002; Manna and Harman 2014; McCulloch et al. 2002).”
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Exercise and the Mitochondria
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“Briefly, a nuclearencoded RNA polymerase similar to those found in T3 and T7 bacteriophages [7] recognizes a promoter on both strands of mtDNA with the aid of two transcription factors [8,9].”
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Transcriptional Control of Striated Muscle Mitochondrial Biogenesis and Function
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“It has been shown that excessive TFAM also causes mitochondrial dysfunction by inhibiting mitochondrial gene expression (Falkenberg et al., 2002; Maniura-Weber et al., 2004; Pohjoismäki et al., 2006; Ylikallio et al., 2010).”
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Overview
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“TFAM, together with other auxiliary factors including mtTFB1 and mtTFB2, promotes the transcription of mtDNA by mitochondrial RNA polymerase (POLRMT) into long polycistronic precursor RNAs, which are further processed into individual RNAs (Chen et al. , 2019; Falkenberg et al., 2002).”
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“Consistent with this interpretation, the mitochondrial RNA polymerase ( POLRMT ) was significantly upregulated in the heart of humans, the mouse, and rat and in human kidney; these findings support the possibility that core mtDNA transcriptional machinery partially participates in the CMNGE upregulation after birth (S…”
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“The key mtDNA transcription factors comprise mitochondrial transcription factors A (TFAM) [ 57 ], B1 (TFB1M), and B2 (TFB2M) [ 58 ], of which TFB2M is deemed to be essential [ 59 ], mitochondrial RNA polymerase (POLRMT) [ 60 ], mitochondrial transcription elongation factor (TEFM) [ 61 ], and…”
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“The use of extracts from different parts of plants in the preparation of therapeutic potions is a more cultural than social attribute (Falkenberg et al., 2002).”
Background
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“Interaction of Tfam with the transcriptional machinery including mt-F1B, mt-F2B and mt-RNA pol leads to a constitutive stimulation of organelle transcription [157, 158], through the synthesis of a polycistronic RNA subsequently cleaved”
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“TFAM is essential both for transcription initiation [15-18] and it is present in mitochondrial nucleoid controlling mtDNA copy number control [19,20].”
BackgroundMethod
The Porcine Mitochondrial Transcription Factor a Gene: Molecular Characterization, Radiation Hybrid Mapping and Genetic Diversity among 12 Pig Breeds
Kunej, T., Wu, X., Michal, J. J., BERLIČ, T. M., Jiang, Z., & Dovč, P. (2009) · American Journal of Animal and Veterinary Sciences · doi:10.3844/ajavsp.2009.129.135 · Cited by 2
“For example, mice heterozygous for a targeted mutation of the Tfam gene exhibited reduced mtDNA copy number, while homozygous knockout embryos exhibited severe mtDNA loss and embryonic lethality, probably due to abolished transcription dependent priming of mtDNA replication [2,3] .”
Background
Chemical Constituents of Clerodendrum splendens (Lamiaceae) and Their Antioxidant Activities
Oscar, N. D. Y. (2018) · Journal of Diseases and Medicinal Plants · doi:10.11648/j.jdmp.20180405.11 · Cited by 2
“The use of extracts of different parts of plants in the preparation of therapeutic potions is a mark more cultural than social [1].”
Background

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.
ORCID: Falkenberg, Maria
Larsson, Nils-Göran
Gustafsson, Claes M.
Venue: Nature Genetics
Publisher: Springer Science and Business Media LLC
Published: 2002-06-17
Volume: 31
Issue: 3
Pages: 289-294
ISSN: 1061-4036 (ISSN-L)
1546-1718
DOI: 10.1038/ng909
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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Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

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