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.
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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
Brain metabolism is abnormal in the mdx model of Duchenne muscular dystrophy
TL;DR: Whether similar abnormalities occur in mdx brain in vivo by using 31P-MRS in addition to metabolite and enzyme analysis to study cerebral metabolism is determined and proposed that these biochemical changes may be a factor in the reduced cognitive capacity of mdx mice and some DMD children.
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Mitochondrial Dysfunction Is an Early Consequence of Partial or Complete Dystrophin Loss in mdx Mice
Timothy M. Moore,Timothy M. Moore,Amanda J. Lin,Alexander R. Strumwasser,Kevin Cory,Kate Whitney,Theodore Ho,Timothy Ho,Joseph L. Lee,Daniel H. Rucker,Christina Q. Nguyen,Aidan Yackly,Sushil K. Mahata,Sushil K. Mahata,Jonathan Wanagat,Linsey Stiles,Lorraine P. Turcotte,Rachelle H. Crosbie,Zhenqi Zhou +18 more
TL;DR: It is suggested that before the onset of myofiber necrosis, mitochondrial and metabolic abnormalities are present within the mdx mouse, which is in line with previous data.
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Gene expression in mdx mouse muscle in relation to age and exercise: aberrant mechanical–metabolic coupling and implications for pre-clinical studies in Duchenne muscular dystrophy
Giulia Maria Camerino,Maria Cannone,Arcangela Giustino,Ada Maria Massari,Roberta Francesca Capogrosso,Anna Cozzoli,Annamaria De Luca +6 more
TL;DR: Chronic exercise with minor adaptive effects in WT muscles leads to maladaptation in mdx muscles with a disequilibrium between protective and damaging signals, leading to increased understanding of the pathways involved in the altered mechanical-metabolic coupling.
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HDAC inhibitors tune miRNAs in extracellular vesicles of dystrophic muscle-resident mesenchymal cells
Martina Sandoná,Silvia Consalvi,Tucciarone L,Marco De Bardi,Manuel Scimeca,Daniela F. Angelini,Valentina Buffa,Adele D'Amico,Enrico Bertini,Sara Cazzaniga,Paolo Bettica,Marina Bouché,Antonella Bongiovanni,Pier Lorenzo Puri,Valentina Saccone +14 more
TL;DR: AntagomiR‐mediated blockade of individual miRs reveals a specific requirement of miR‐206 for EV‐induced expansion of MuSCs and regeneration of dystrophic muscles, and indicates that cooperative activity of HDACi‐induced miRs accounts for the net biological effect of these EVs.
Journal ArticleDOI
Autophagy balances mtDNA synthesis and degradation by DNA polymerase POLG during starvation.
TL;DR: This study reveals that mitochondria rely on the homeostatic functions of autophagy to balance synthetic and degradative modes of POLG, which control copy number dynamics and stability of the mitochondrial genome.