The DOI matches its Crossref record; 16 of the 16 references it cites were found in a registry or an index.
Maintenance and Expression of Mammalian Mitochondrial DNA
Abstract
Mammalian mitochondrial DNA (mtDNA) encodes 13 proteins that are essential for the function of the oxidative phosphorylation system, which is composed of four respiratory-chain complexes and adenosine triphosphate (ATP) synthase. Remarkably, the maintenance and expression of mtDNA depend on the mitochondrial import of hundreds of nuclear-encoded proteins that control genome maintenance, replication, transcription, RNA maturation, and mitochondrial translation. The importance of this complex regulatory system is underscored by the identification of numerous mutations of nuclear genes that impair mtDNA maintenance and expression at different levels, causing human mitochondrial diseases with pleiotropic clinical manifestations. The basic scientific understanding of the mechanisms controlling mtDNA function has progressed considerably during the past few years, thanks to advances in biochemistry, genetics, and structural biology. The challenges for the future will be to understand how mtDNA maintenance and expression are regulated and to what extent direct intramitochondrial cross talk between different processes, such as transcription and translation, is important.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Premature ageing in mice expressing defective mitochondrial DNA polymerase
Trifunovic, A., Wredenberg, A., Falkenberg, M., Spelbrink, J. N., Rovio, A. T., Bruder, C. E., Bohlooly-Y, M., Gidlöf, S., Oldfors, A., Wibom, R., et al. (2004) · Nature · doi:10.1038/nature02517 · Cited by 2,734
Self-citationSpelbrink, J. N., Li, F.-Y., Tiranti, V., Nikali, K., Yuan, Q.-P., Tariq, M., Wanrooij, S., Garrido, N., Comi, G., Morandi, L., et al. (2001) · Nature Genetics · doi:10.1038/90058 · Cited by 882
Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA
Falkenberg, M., Gaspari, M., Rantanen, A., Trifunovic, A., Larsson, N.-G., & Gustafsson, C. M. (2002) · Nature Genetics · doi:10.1038/ng909 · Cited by 598
Self-citationDNA Polymerase γ, The Mitochondrial Replicase
Kaguni, L. S. (2004) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.72.121801.161455 · Cited by 414
Korhonen, J. A., Gaspari, M., & Falkenberg, M. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m306981200 · Cited by 298
Mitochondrial DNA nucleoid structure
Bogenhagen, D. F. (2012) · Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms · doi:10.1016/j.bbagrm.2011.11.005 · Cited by 290
Germline mitochondrial DNA mutations aggravate ageing and can impair brain development
Ross, J. M., Stewart, J. B., Hagström, E., Brené, S., Mourier, A., Coppotelli, G., Freyer, C., Lagouge, M., Hoffer, B. J., Olson, L., et al. (2013) · Nature · doi:10.1038/nature12474 · Cited by 273
Self-citationMitochondrial DNA polymerase gamma is essential for mammalian embryogenesis
Hance, N., Ekstrand, M. I., & Trifunovic, A. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi184 · Cited by 272
Longley, M. J., Nguyen, D., Kunkel, T. A., & Copeland, W. C. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m105230200 · Cited by 270
Twinkle helicase is essential for mtDNA maintenance and regulates mtDNA copy number
Tyynismaa, H., Sembongi, H., Bokori-Brown, M., Granycome, C., Ashley, N., Poulton, J., Jalanko, A., Spelbrink, J. N., Holt, I. J., & Suomalainen, A. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh342 · Cited by 250
Cited by
Fluorescence nanoscopy in cell biology
Sahl, S. J., Hell, S. W., & Jakobs, S. (2017) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2017.71 · Cited by 990
Mitochondrial diseases: the contribution of organelle stress responses to pathology
Suomalainen, A., & Battersby, B. J. (2018) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2017.66 · Cited by 505
Quality control of the mitochondrion
Ng, M. Y. W., Wai, T., & Simonsen, A. (2021) · Developmental Cell · doi:10.1016/j.devcel.2021.02.009 · Cited by 445
Mitochondria Know No Boundaries: Mechanisms and Functions of Intercellular Mitochondrial Transfer
Torralba, D., Baixauli, F., & Sánchez-Madrid, F. (2016) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2016.00107 · Cited by 440
Mechanisms and regulation of protein synthesis in mitochondria
Kummer, E., & Ban, N. (2021) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-021-00332-2 · Cited by 349
Metadata
- Authors
- Gustafsson, C. M., Falkenberg, M., & Larsson, N.-G.
- Venue
- Annual Review of Biochemistry
- Publisher
- Annual Reviews
- Published
- 2016-06-02
- Volume
- 85
- Issue
- 1
- Pages
- 133-160
- ISSN
- 0066-4154 (ISSN-L)
- 1545-4509
- OpenAlex
- W2215582843
- Cited by
- 700
- References
- 16
- FWCI
- 3.98
- Type
- journal article
- Language
- en
- Open access
- closed
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
How to cite
Cite CoChat for the checks on this page, and the paper for its own findings.
- This page
- CoChat. (2026). Citation check: Maintenance and Expression of Mammalian Mitochondrial DNA. https://app.cochat.ai/papers/10.1146/annurev-biochem-060815-014402
- The paper
- Gustafsson, C. M., Falkenberg, M., & Larsson, N.-G. (2016). Maintenance and Expression of Mammalian Mitochondrial DNA. Annual Review of Biochemistry. https://doi.org/10.1146/annurev-biochem-060815-014402
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
