The mitochondrial permeability transition pore: channel formation by F-ATP synthase, integration in signal transduction, and role in pathophysiology

P Bernardi, A Rasola, M Forte… - Physiological …, 2015 - journals.physiology.org
The mitochondrial permeability transition (PT) is a permeability increase of the inner
mitochondrial membrane mediated by a channel, the permeability transition pore (PTP) …

Muscular dystrophies

E Mercuri, CG Bönnemann, F Muntoni - The Lancet, 2019 - thelancet.com
Muscular dystrophies are primary diseases of muscle due to mutations in more than 40
genes, which result in dystrophic changes on muscle biopsy. Now that most of the genes …

Autophagy is required to maintain muscle mass

E Masiero, L Agatea, C Mammucari, B Blaauw, E Loro… - Cell metabolism, 2009 - cell.com
The ubiquitin-proteasome and autophagy-lysosome pathways are the two major routes for
protein and organelle clearance. In skeletal muscle, both systems are under FoxO regulation …

[HTML][HTML] Mitochondrial pharmacology

RAJ Smith, RC Hartley, HM Cocheme… - Trends in pharmacological …, 2012 - cell.com
Mitochondria are being recognized as key factors in many unexpected areas of biomedical
science. In addition to their well-known roles in oxidative phosphorylation and metabolism, it …

Monogenic mitochondrial disorders

WJH Koopman, PHGM Willems… - New England Journal …, 2012 - Mass Medical Soc
Monogenic Mitochondrial Disorders | New England Journal of Medicine Skip to main content The
New England Journal of Medicine homepage Advanced Search SEARCH SPECIALTIES …

Immune-mediated pathology in Duchenne muscular dystrophy

AS Rosenberg, M Puig, K Nagaraju… - Science translational …, 2015 - science.org
Immunological and inflammatory processes downstream of dystrophin deficiency as well as
metabolic abnormalities, defective autophagy, and loss of regenerative capacity all …

Linking oxidative stress and DNA damage to changes in the expression of extracellular matrix components

SG Martins, R Zilhão, S Thorsteinsdóttir… - Frontiers in …, 2021 - frontiersin.org
Cells are subjected to endogenous [eg, reactive oxygen species (ROS), replication stress]
and exogenous insults (eg, UV light, ionizing radiation, and certain chemicals), which can …

[HTML][HTML] Mitochondrial channels: ion fluxes and more

I Szabo, M Zoratti - Physiological reviews, 2014 - journals.physiology.org
The field of mitochondrial ion channels has recently seen substantial progress, including the
molecular identification of some of the channels. An integrative approach using genetics …

Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration

P Grumati, L Coletto, P Sabatelli, M Cescon… - Nature medicine, 2010 - nature.com
Autophagy is crucial in the turnover of cell components, and clearance of damaged
organelles by the autophagic-lysosomal pathway is essential for tissue homeostasis …

Mitochondrial permeability transition: a molecular lesion with multiple drug targets

T Briston, DL Selwood, G Szabadkai… - Trends in pharmacological …, 2019 - cell.com
Mitochondrial permeability transition, as the consequence of opening of a mitochondrial
permeability transition pore (mPTP), is a cellular catastrophe. Initiating bioenergetic collapse …