Mitochondria at the crossroads of health and disease

A Suomalainen, J Nunnari - Cell, 2024 - cell.com
Mitochondria reside at the crossroads of catabolic and anabolic metabolism—the essence of
life. How their structure and function are dynamically tuned in response to tissue-specific …

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 …

Distinct fission signatures predict mitochondrial degradation or biogenesis

T Kleele, T Rey, J Winter, S Zaganelli, D Mahecic… - Nature, 2021 - nature.com
Mitochondrial fission is a highly regulated process that, when disrupted, can alter
metabolism, proliferation and apoptosis,–. Dysregulation has been linked to …

The role of mitochondria in neurodegenerative diseases: the lesson from Alzheimer's disease and Parkinson's disease

G Monzio Compagnoni, A Di Fonzo, S Corti… - Molecular …, 2020 - Springer
Although the pathogenesis of neurodegenerative diseases is still widely unclear, various
mechanisms have been proposed and several pieces of evidence are supportive for an …

Niacin cures systemic NAD+ deficiency and improves muscle performance in adult-onset mitochondrial myopathy

E Pirinen, M Auranen, NA Khan, V Brilhante, N Urho… - Cell metabolism, 2020 - cell.com
NAD+ is a redox-active metabolite, the depletion of which has been proposed to promote
aging and degenerative diseases in rodents. However, whether NAD+ depletion occurs in …

POLG-related disorders and their neurological manifestations

S Rahman, WC Copeland - Nature Reviews Neurology, 2019 - nature.com
The POLG gene encodes the mitochondrial DNA polymerase that is responsible for
replication of the mitochondrial genome. Mutations in POLG can cause early childhood …

Mitochondrial diseases

GS Gorman, PF Chinnery, S DiMauro… - Nature reviews Disease …, 2016 - nature.com
Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …

Mitochondrial diseases: the contribution of organelle stress responses to pathology

A Suomalainen, BJ Battersby - Nature reviews Molecular cell biology, 2018 - nature.com
Mitochondrial diseases affect one in 2,000 individuals; they can present at any age and they
can manifest in any organ. How defects in mitochondria can cause such a diverse range of …

The alternative reality of plant mitochondrial DNA: One ring does not rule them all

A Kozik, BA Rowan, D Lavelle, L Berke… - PLoS …, 2019 - journals.plos.org
Plant mitochondrial genomes are usually assembled and displayed as circular maps based
on the widely-held view across the broad community of life scientists that circular 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 …