Mechanisms and regulation of human mitochondrial transcription

BG Tan, CM Gustafsson, M Falkenberg - Nature Reviews Molecular Cell …, 2024 - nature.com
The expression of mitochondrial genes is regulated in response to the metabolic needs of
different cell types, but the basic mechanisms underlying this process are still poorly …

Mitochondrial DNA mutations in human disease

RW Taylor, DM Turnbull - Nature Reviews Genetics, 2005 - nature.com
The human mitochondrial genome is extremely small compared with the nuclear genome,
and mitochondrial genetics presents unique clinical and experimental challenges. Despite …

The significance of mitochondrial dysfunction in cancer

Y Luo, J Ma, W Lu - International journal of molecular sciences, 2020 - mdpi.com
As an essential organelle in nucleated eukaryotic cells, mitochondria play a central role in
energy metabolism, maintenance of redox balance, and regulation of apoptosis …

Transcriptional paradigms in mammalian mitochondrial biogenesis and function

RC Scarpulla - Physiological reviews, 2008 - journals.physiology.org
Mitochondria contain their own genetic system and undergo a unique mode of cytoplasmic
inheritance. Each organelle has multiple copies of a covalently closed circular DNA genome …

Maintenance and expression of mammalian mitochondrial DNA

CM Gustafsson, M Falkenberg… - Annual review of …, 2016 - annualreviews.org
Mammalian mitochondrial DNA (mtDNA) encodes 13 proteins that are essential for the
function of the oxidative phosphorylation system, which is composed of four respiratory …

The role of mitochondria in acute kidney injury and chronic kidney disease and its therapeutic potential

X Zhang, E Agborbesong, X Li - International journal of molecular …, 2021 - mdpi.com
Mitochondria are heterogeneous and highly dynamic organelles, playing critical roles in
adenosine triphosphate (ATP) synthesis, metabolic modulation, reactive oxygen species …

Transcriptional regulatory circuits controlling mitochondrial biogenesis and function

DP Kelly, RC Scarpulla - Genes & development, 2004 - genesdev.cshlp.org
We are witnessing a period of renewed interest in the biology of the mitochondrion. The
mitochondrion serves a critical function in the maintenance of cellular energy stores …

Small-molecule inhibitors of human mitochondrial DNA transcription

NA Bonekamp, B Peter, HS Hillen, A Felser… - Nature, 2020 - nature.com
Altered expression of mitochondrial DNA (mtDNA) occurs in ageing and a range of human
pathologies (for example, inborn errors of metabolism, neurodegeneration and cancer) …

Transcriptional control of mitochondrial biogenesis: the central role of PGC-1α

R Ventura-Clapier, A Garnier… - Cardiovascular …, 2008 - academic.oup.com
Although the concept of energy starvation in the failing heart was proposed decades ago,
still very little is known about the origin of energetic failure. Recent advances in molecular …

Mitochondrial transcription and translation: overview

AR D'Souza, M Minczuk - Essays in biochemistry, 2018 - portlandpress.com
Mitochondria are the major source of ATP in the cell. Five multi-subunit complexes in the
inner membrane of the organelle are involved in the oxidative phosphorylation required for …