The DOI matches its Crossref record; 220 of the 220 references it cites were found in a registry or an index.
The mitochondrial genome: structure, transcription, translation and replication
Open accessAbstract
Mitochondria play a central role in cellular energy provision. The organelles contain their own genome with a modified genetic code. The mammalian mitochondrial genome is transmitted exclusively through the female germ line. The human mitochondrial DNA (mtDNA) is a double-stranded, circular molecule of 16569 bp and contains 37 genes coding for two rRNAs, 22 tRNAs and 13 polypeptides. The mtDNA-encoded polypeptides are all subunits of enzyme complexes of the oxidative phosphorylation system. Mitochondria are not self-supporting entities but rely heavily for their functions on imported nuclear gene products. The basic mechanisms of mitochondrial gene expression have been solved. Cis-acting mtDNA sequences have been characterised by sequence comparisons, mapping studies and mutation analysis both in vitro and in patients harbouring mtDNA mutations. Characterisation of trans-acting factors has proven more difficult but several key enzymes involved in mtDNA replication, transcription and protein synthesis have now been biochemically identified and some have been cloned. These studies revealed that, although some factors may have an additional function elsewhere in the cell, most are unique to mitochondria. It is expected that cell cultures of patients with mitochondrial diseases will increasingly be used to address fundamental questions about mtDNA expression.
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“Typically located in the inner mitochondrial matrix, mtDNA encodes for 37 genes: 13 mRNAs that are translated into subunits of components of the oxidative phosphorylation system, 2 ribosomal RNAs (rRNA) and 22 transfer RNAs (tRNAs) (Taanman, 1999).”
From Cytoplasmic DNA: sources, sensing, and role in aging and disease (2021) · cites this paper as Taanman, 1999
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“Unlike other organelles in cells of the human body, mitochondria harbor a small amount of their own DNA (mtDNA), which encodes a series of critical proteins involved in mitochondrial respiration, including 13 genes encoding subunits of the respiratory chain, two genes encoding ribosomal RNAs, and 22 genes encoding tra…”
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Metadata
- Authors
- Taanman, J.-W.
- Affiliations
- University of London (GB)
- Corresponding author
- Taanman, Jan-Willem
- ORCID
- Taanman, Jan-Willem
- Venue
- Biochimica et Biophysica Acta (BBA) - Bioenergetics
- Publisher
- Elsevier BV
- Published
- 1999-02
- Volume
- 1410
- Issue
- 2
- Pages
- 103-123
- ISSN
- 0005-2728 (ISSN-L)
- OpenAlex
- W2038481472
- Cited by
- 1,635
- References
- 220
- Citations by year
- 2026: 57 · 2025: 89 · 2024: 101 · 2023: 132 · 2022: 113 · 2021: 126 · 2020: 89 · 2019: 122 · 2018: 105 · 2017: 92 · 2016: 69 · 2015: 52 · 2014: 57 · 2013: 47 · 2012: 37
- FWCI
- 1.56
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Biochimica et Biophysica Acta (BBA) - Bioenergetics (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Wellcome Trust (048410)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › ATP Synthase and ATPases Research
- Keywords
- mitochondrial DNA, mitochondrial genome structure, mitochondrial transcription, mitochondrial translation, mitochondrial DNA replication, oxidative phosphorylation, mitochondrial gene expression, mitochondrial DNA mutations, cis-regulatory elements, trans-acting factors, mitochondrial disease, maternal inheritance, mitochondrial protein import
- MeSH terms
- Cells, Cultured, DNA Replication, DNA, Mitochondrial, Humans, Transcription, Genetic, Protein Biosynthesis, Molecular Structure
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