Mitochondrial dynamics and its involvement in disease

DC Chan - Annual review of pathology: mechanisms of disease, 2020‏ - annualreviews.org
The dynamic properties of mitochondria—including their fusion, fission, and degradation—
are critical for their optimal function in energy generation. The interplay of fusion and fission …

Mitochondrial DNA copy number in human disease: the more the better?

R Filograna, M Mennuni, D Alsina, NG Larsson - FEBS letters, 2021‏ - Wiley Online Library
Most of the genetic information has been lost or transferred to the nucleus during the
evolution of mitochondria. Nevertheless, mitochondria have retained their own genome that …

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 fusion, fission, and mitochondrial toxicity

JN Meyer, TC Leuthner, AL Luz - Toxicology, 2017‏ - Elsevier
Mitochondrial dynamics are regulated by two sets of opposed processes: mitochondrial
fusion and fission, and mitochondrial biogenesis and degradation (including mitophagy), as …

Disturbed mitochondrial dynamics and neurodegenerative disorders

F Burté, V Carelli, PF Chinnery… - Nature reviews …, 2015‏ - nature.com
Mitochondria form a highly interconnected tubular network throughout the cell via a dynamic
process, with mitochondrial segments fusing and breaking apart continuously. Strong …

[HTML][HTML] Mitochondrial DNA maintenance defects

AW El-Hattab, WJ Craigen, F Scaglia - Biochimica et Biophysica Acta (BBA) …, 2017‏ - Elsevier
The maintenance of mitochondrial DNA (mtDNA) depends on a number of nuclear gene-
encoded proteins including a battery of enzymes forming the replisome needed to …

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

S Bannwarth, S Ait-El-Mkadem, A Chaussenot… - Brain, 2014‏ - academic.oup.com
Mitochondrial DNA instability disorders are responsible for a large clinical spectrum, among
which amyotrophic lateral sclerosis-like symptoms and frontotemporal dementia are …

Mitochondria, oxidative stress and neurodegeneration

A Federico, E Cardaioli, P Da Pozzo, P Formichi… - Journal of the …, 2012‏ - Elsevier
Mitochondria are involved in ATP supply to cells through oxidative phosphorylation
(OXPHOS), synthesis of key molecules and response to oxidative stress, as well as in …

Mitochondrial fusion/fission dynamics in neurodegeneration and neuronal plasticity

AM Bertholet, T Delerue, AM Millet, MF Moulis… - Neurobiology of …, 2016‏ - Elsevier
Mitochondria are dynamic organelles that continually move, fuse and divide. The dynamic
balance of fusion and fission of mitochondria determines their morphology and allows their …

Mitochondria as a target of environmental toxicants

JN Meyer, MCK Leung, JP Rooney… - toxicological …, 2013‏ - academic.oup.com
Enormous strides have recently been made in our understanding of the biology and
pathobiology of mitochondria. Many diseases have been identified as caused by …