[HTML][HTML] Mitochondrial diseases: hope for the future

OM Russell, GS Gorman, RN Lightowlers, DM Turnbull - Cell, 2020 - cell.com
Mitochondrial diseases are clinically heterogeneous disorders caused by a wide spectrum
of mutations in genes encoded by either the nuclear or the mitochondrial genome …

Mitochondrial spare respiratory capacity: mechanisms, regulation, and significance in non‐transformed and cancer cells

P Marchetti, Q Fovez, N Germain, R Khamari… - The FASEB …, 2020 - Wiley Online Library
Mitochondrial metabolism must constantly adapt to stress conditions in order to maintain
bioenergetic levels related to cellular functions. This absence of proper adaptation can be …

Accumulation of succinate controls activation of adipose tissue thermogenesis

EL Mills, KA Pierce, MP Jedrychowski, R Garrity… - Nature, 2018 - nature.com
Thermogenesis by brown and beige adipose tissue, which requires activation by external
stimuli, can counter metabolic disease. Thermogenic respiration is initiated by adipocyte …

pH-gated succinate secretion regulates muscle remodeling in response to exercise

A Reddy, LHM Bozi, OK Yaghi, EL Mills, H Xiao… - Cell, 2020 - cell.com
In response to skeletal muscle contraction during exercise, paracrine factors coordinate
tissue remodeling, which underlies this healthy adaptation. Here we describe a pH-sensing …

Principles and functions of metabolic compartmentalization

L Bar-Peled, N Kory - Nature metabolism, 2022 - nature.com
Metabolism has historically been studied at the levels of whole cells, whole tissues and
whole organisms. As a result, our understanding of how compartmentalization—the spatial …

Why succinate? Physiological regulation by a mitochondrial coenzyme Q sentinel

MP Murphy, ET Chouchani - Nature chemical biology, 2022 - nature.com
Metabolites once considered solely in catabolism or anabolism turn out to have key
regulatory functions. Among these, the citric acid cycle intermediate succinate stands out …

A smart theranostic prodrug system activated by reactive oxygen species for regional chemotherapy of metastatic cancer

L Liu, F Liu, D Liu, W Yuan, M Zhang… - Angewandte …, 2022 - Wiley Online Library
Metastatic cancer is difficult to cure because of its uncontrollable nature and side effects
during treatment. We constructed a reactive oxygen species (ROS)‐activated smart …

Leigh syndrome: a tale of two genomes

AB Bakare, EJ Lesnefsky, S Iyer - Frontiers in Physiology, 2021 - frontiersin.org
Leigh syndrome is a rare, complex, and incurable early onset (typically infant or early
childhood) mitochondrial disorder with both phenotypic and genetic heterogeneity. The …

Succinate accumulation drives ischaemia-reperfusion injury during organ transplantation

JL Martin, ASH Costa, AV Gruszczyk, TE Beach… - Nature …, 2019 - nature.com
During heart transplantation, storage in cold preservation solution is thought to protect the
organ by slowing metabolism, providing osmotic support and minimizing ischaemia …

Preventing mitochondrial reverse electron transport as a strategy for cardioprotection

HA Prag, MP Murphy, T Krieg - Basic Research in Cardiology, 2023 - Springer
In the context of myocardial infarction, the burst of superoxide generated by reverse electron
transport (RET) at complex I in mitochondria is a crucial trigger for damage during …