Journal ArticleDOI
Givinostat as metabolic enhancer reverting mitochondrial biogenesis deficit in Duchenne Muscular Dystrophy.
Matteo Giovarelli,Silvia Zecchini,Giorgia Catarinella,Claudia Moscheni,Patrizia Sartori,Cecilia Barbieri,Paulina Roux-Biejat,Alessandra Napoli,Chiara Vantaggiato,Davide Cervia,Cristiana Perrotta,Emilio Clementi,Emilio Clementi,Lucia Latella,Clara De Palma +14 more
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TLDR
In this paper, Givinostat was shown to positively modify the epigenetic profile of peroxisome proliferator-activated receptor-gamma coactivator 1 α (PGC-1α) promoter, sustaining mitochondrial biogenesis and oxidative fiber type switch.About:
This article is published in Pharmacological Research.The article was published on 2021-08-01. It has received 12 citations till now. The article focuses on the topics: Mitochondrial biogenesis & Duchenne muscular dystrophy.read more
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Journal ArticleDOI
Alisporivir Improves Mitochondrial Function in Skeletal Muscle of mdx Mice but Suppresses Mitochondrial Dynamics and Biogenesis.
Mikhail V. Dubinin,Vlada S. Starinets,Eugeny Yu. Talanov,I. B. Mikheeva,Natalia V. Belosludtseva,Konstantin N. Belosludtsev +5 more
TL;DR: In this article, the effect of intraperitoneal administration of a non-immunosuppressive inhibitor of calcium-dependent mitochondrial permeability transition (MPT) pore alisporivir on the state of skeletal muscles and the functioning of mitochondria in dystrophin-deficient mdx mice was studied.
Journal ArticleDOI
BKCa Activator NS1619 Improves the Structure and Function of Skeletal Muscle Mitochondria in Duchenne Dystrophy
Mikhail V. Dubinin,Vlada S. Starinets,Natalia V. Belosludtseva,I. B. Mikheeva,Yuliya A Chelyadnikova,Anastasia D Igoshkina,Aliya B. Vafina,A. A. Vedernikov,Konstantin N. Belosludtsev +8 more
TL;DR: In this paper , the role of the BKCa activator NS1619 in the development of Duchenne muscular dystrophy (DMD) was examined in dystrophin-deficient mdx mice.
Journal ArticleDOI
Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study
Matteo Giovarelli,Francesca Arnaboldi,Silvia Zecchini,Laura Cornaghi,Ambra Nava,Michele Sommariva,Emilio Clementi,Nicoletta Gagliano +7 more
TL;DR: Fibrosis mostly affects diaphragm and quadriceps with a higher collagen cross-linking and inhibition of MMPs that contribute differently to progressive collagen accumulation during fibrotic remodelling, which may provide new targets for tailored therapeutic interventions.
Journal ArticleDOI
The Effect of Uridine on the State of Skeletal Muscles and the Functioning of Mitochondria in Duchenne Dystrophy
Mikhail V. Dubinin,Vlada S. Starinets,Natalia V. Belosludtseva,I. B. Mikheeva,Yuliya A Chelyadnikova,Daria K. Penkina,A. A. Vedernikov,Konstantin N. Belosludtsev +7 more
TL;DR: It is found that chronic uridine administration reduced fibrosis in the skeletal muscles of mdx mice, but it had no effect on the intensity of degeneration/regeneration cycles and inflammation, pseudohypetrophy, and muscle strength of the animals.
Journal ArticleDOI
Ion Channels of the Sarcolemma and Intracellular Organelles in Duchenne Muscular Dystrophy: A Role in the Dysregulation of Ion Homeostasis and a Possible Target for Therapy
TL;DR: In this paper , a review is devoted to the analysis of current data on changes in the structure, functioning, and regulation of the activity of ion channels in striated muscles in DMD and their contribution to the disruption of muscle function and the development of pathology.
References
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Journal ArticleDOI
Muscle-specific Drp1 overexpression impairs skeletal muscle growth via translational attenuation
Thierry Touvier,C. De Palma,Elena Rigamonti,Alessandra Scagliola,Elena Incerti,L. Mazelin,J. L. Thomas,M. D'Antonio,Letterio S. Politi,Laurent Schaeffer,Emilio Clementi,Silvia Brunelli +11 more
TL;DR: It is found that Drp1 overexpression activates the stress-induced PKR/eIF2α/Fgf21 pathway thus leading to an attenuated protein synthesis and downregulation of the growth hormone pathway, revealing for the first time how mitochondrial network dynamics influence muscle growth.
Journal ArticleDOI
Hypertrophic response of Duchenne and limb-girdle muscular dystrophies is associated with activation of Akt pathway.
TL;DR: It is proposed that Akt may serve as an early biomarker of disease and thatAkt activation mediates hypertrophy in muscular dystrophy and how early modification of Akt activity influences disease pathogenesis is investigated.
Journal ArticleDOI
Multiple pathological events in exercised dystrophic mdx mice are targeted by pentoxifylline: outcome of a large array of in vivo and ex vivo tests
Rosa Burdi,Jean François Rolland,Bodvael Fraysse,Litvinova Ks,Anna Cozzoli,Viviana Giannuzzi,Antonella Liantonio,Giulia Maria Camerino,Valeriana Sblendorio,Roberta Francesca Capogrosso,Beniamino Palmieri,Francesca Andreetta,Paolo Confalonieri,Leonarda De Benedictis,Monica Montagnani,Annamaria De Luca +15 more
TL;DR: In vivo and ex vivo, pentoxifylline restored the mechanical threshold, an electrophysiological index of calcium homeostasis, and reduced resting cytosolic calcium in extensor digitorum longus muscle fibers and allows insight in the level of cross talk between pathogenetic events in workloaded dystrophic muscle.
Journal ArticleDOI
Resveratrol induces expression of the slow, oxidative phenotype in mdx mouse muscle together with enhanced activity of the SIRT1-PGC-1α axis
TL;DR: This study demonstrates that RSV can stimulate SIRT1 and PGC-1α activation, which in turn may promote expression of the slow, oxidative myogenic program in mdx mouse muscle, and highlights the importance of selecting an appropriate dosage regimen of RSV to maximize its potential therapeutic effectiveness for future application in DMD patients.
Journal ArticleDOI
Early myopathy in Duchenne muscular dystrophy is associated with elevated mitochondrial H 2 O 2 emission during impaired oxidative phosphorylation.
Meghan C. Hughes,Sofhia V. Ramos,Patrick C. Turnbull,Irena A. Rebalka,Andrew W. Cao,Cynthia M. F. Monaco,Nina E. Varah,Brittany A. Edgett,Jason S. Huber,Peyman Tadi,Luca J. Delfinis,Uwe Schlattner,Jeremy A. Simpson,Thomas J. Hawke,Christopher G. R. Perry +14 more
TL;DR: This study performed a systematic evaluation of the nature and degree of mitochondrial‐derived H2O2 emission and mitochondrial oxidative dysfunction in a mouse model of DMD by designing in vitro bioenergetic assessments that attempt to mimic in vivo conditions known to be critical for the regulation of mitochondrial bioenergetics.