Fundamental mechanisms of regulated cell death and implications for heart disease

DP Del Re, D Amgalan, A Linkermann… - Physiological …, 2019 - journals.physiology.org
Twelve regulated cell death programs have been described. We review in detail the basic
biology of nine including death receptor-mediated apoptosis, death receptor-mediated …

Mitophagy in human diseases

L Doblado, C Lueck, C Rey, AK Samhan-Arias… - International journal of …, 2021 - mdpi.com
Mitophagy is a selective autophagic process, essential for cellular homeostasis, that
eliminates dysfunctional mitochondria. Activated by inner membrane depolarization, it plays …

Inhibition of ATP synthase reverse activity restores energy homeostasis in mitochondrial pathologies

R Acin‐Perez, C Benincá, L Fernandez del Rio… - The EMBO …, 2023 - embopress.org
The maintenance of cellular function relies on the close regulation of adenosine
triphosphate (ATP) synthesis and hydrolysis. ATP hydrolysis by mitochondrial ATP Synthase …

Mitochondria-targeted drugs

RA Zinovkin, AA Zamyatnin - Current Molecular Pharmacology, 2019 - ingentaconnect.com
Background: Targeting of drugs to the subcellular compartments represents one of the
modern trends in molecular pharmacology. The approach for targeting mitochondria was …

Urolithin A improves muscle function by inducing mitophagy in muscular dystrophy

P Luan, D D'Amico, PA Andreux, PP Laurila… - Science translational …, 2021 - science.org
Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy, and despite
advances in genetic and pharmacological disease-modifying treatments, its management …

Signaling pathways regulating the fate of fibro/adipogenic progenitors (FAPs) in skeletal muscle regeneration and disease

G Giuliani, M Rosina, A Reggio - The FEBS journal, 2022 - Wiley Online Library
The characterization of fibro/adipogenic progenitor cells (FAPs) in the skeletal muscle has
contributed to modify the monocentric view of muscle regeneration beyond muscle satellite …

[HTML][HTML] TGF-β–driven muscle degeneration and failed regeneration underlie disease onset in a DMD mouse model

DAG Mázala, JS Novak, MW Hogarth, M Nearing… - JCI insight, 2020 - ncbi.nlm.nih.gov
Duchenne muscular dystrophy (DMD) is a chronic muscle disease characterized by poor
myogenesis and replacement of muscle by extracellular matrix. Despite the shared genetic …

The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction

DGS Wilson, A Tinker, T Iskratsch - Communications Biology, 2022 - nature.com
Dystrophin is the central protein of the dystrophin-glycoprotein complex (DGC) in skeletal
and heart muscle cells. Dystrophin connects the actin cytoskeleton to the extracellular matrix …

Mitochondrial redox signaling enables repair of injured skeletal muscle cells

A Horn, JH Van der Meulen, A Defour, M Hogarth… - Science …, 2017 - science.org
Strain and physical trauma to mechanically active cells, such as skeletal muscle myofibers,
injures their plasma membranes, and mitochondrial function is required for their repair. We …

Dysregulation of mitochondrial Ca2+ uptake and sarcolemma repair underlie muscle weakness and wasting in patients and mice lacking MICU1

V Debattisti, A Horn, R Singh, EL Seifert, MW Hogarth… - Cell reports, 2019 - cell.com
Muscle function is regulated by Ca 2+, which mediates excitation-contraction coupling,
energy metabolism, adaptation to exercise, and sarcolemmal repair. Several of these …