Organization and expression of the mammalian mitochondrial genome

O Rackham, A Filipovska - Nature Reviews Genetics, 2022 - nature.com
The mitochondrial genome encodes core subunits of the respiratory chain that drives
oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in …

Mechanisms of replication and repair in mitochondrial DNA deletion formation

GA Fontana, HL Gahlon - Nucleic acids research, 2020 - academic.oup.com
Deletions in mitochondrial DNA (mtDNA) are associated with diverse human pathologies
including cancer, aging and mitochondrial disorders. Large-scale deletions span kilobases …

[HTML][HTML] Oxidized DNA fragments exit mitochondria via mPTP-and VDAC-dependent channels to activate NLRP3 inflammasome and interferon signaling

H Xian, K Watari, E Sanchez-Lopez, J Offenberger… - Immunity, 2022 - cell.com
Mitochondrial DNA (mtDNA) escaping stressed mitochondria provokes inflammation via
cGAS-STING pathway activation and, when oxidized (Ox-mtDNA), it binds cytosolic NLRP3 …

Adaptation to mitochondrial stress requires CHOP-directed tuning of ISR

S Kaspar, C Oertlin, K Szczepanowska, A Kukat… - Science …, 2021 - science.org
In response to disturbed mitochondrial gene expression and protein synthesis, an adaptive
transcriptional response sharing a signature of the integrated stress response (ISR) is …

The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication

M Jiang, X Xie, X Zhu, S Jiang, D Milenkovic, J Misic… - Science …, 2021 - science.org
We report a role for the mitochondrial single-stranded DNA binding protein (mtSSB) in
regulating mitochondrial DNA (mtDNA) replication initiation in mammalian mitochondria …

Mitochondrial function in development and disease

MP Rossmann, SM Dubois… - Disease models & …, 2021 - journals.biologists.com
Mitochondria are organelles with vital functions in almost all eukaryotic cells. Often
described as the cellular 'powerhouses' due to their essential role in aerobic oxidative …

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

E Silva Ramos, E Motori, C Brüser, I Kühl… - PLoS …, 2019 - journals.plos.org
Mitochondrial dynamics is an essential physiological process controlling mitochondrial
content mixing and mobility to ensure proper function and localization of mitochondria at …

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo

NA Bonekamp, M Jiang, E Motori… - Life Science …, 2021 - life-science-alliance.org
Mitochondrial transcription factor A (TFAM) is compacting mitochondrial DNA (dmtDNA) into
nucleoids and directly controls mtDNA copy number. Here, we show that the TFAM-to …

Replication and transcription of human mitochondrial DNA

M Falkenberg, NG Larsson… - Annual Review of …, 2024 - annualreviews.org
Mammalian mitochondrial DNA (mtDNA) is replicated and transcribed by phage-like DNA
and RNA polymerases, and our understanding of these processes has progressed …

Mammalian mitochondrial DNA replication and mechanisms of deletion formation

M Falkenberg, CM Gustafsson - Critical Reviews in Biochemistry …, 2020 - Taylor & Francis
Mammalian mitochondria contain multiple copies of a circular, double-stranded DNA
genome (mtDNA) that codes for subunits of the oxidative phosphorylation machinery …