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
Mitochondrial content is preserved throughout disease progression in the mdx mouse model of Duchenne muscular dystrophy, regardless of taurine supplementation
TL;DR: It is elucidated that taurine affords no significant benefit to mitochondrial content or dynamics in the mdx mouse at either 28 or 70 days, and mitochondrial content and dynamics are not reduced regardless of disease severity.
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Impaired substrate utilization in mitochondria from strain 129 dystrophic mice
TL;DR: It is concluded that skeletal muscle mitochondria from strain 129 dystrophic mice possess impairments in substrate utilization which may result from an abnormality in the transfer of electrons on the substrate side of coenzyme Q in the case of succinate oxidation; a defect on the path of electron flow from NADH to cytochrome c, and a deficiency of NAD+-linked substrates.
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Studies on mitochondria from dystrophic skeletal muscle of mice.
TL;DR: It is found that there is an advantage in having a low concentration of proteinase and EGTA present in the medium during preparation of mitochondria by centrifugation fractionation to obtain the maximum respiration control ratio (RCR) and adenosine diphosphate/oxygen (ADP/O) ratio from isolated muscle mitochondria.
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Diverse Action of Selected Statins on Skeletal Muscle Cells-An Attempt to Explain the Protective Effect of Geranylgeraniol (GGOH) in Statin-Associated Myopathy (SAM).
TL;DR: It has been unambiguously revealed that the mitochondria of C2C12 cells are not sensitive to SIM, although ATR effectively inhibits mitochondrial respiration, and it appears that the calpain inhibitor, N-Acetyl-L-leucyl- L-leunucinal (ALLM), restored the viability that was reduced by ATR and SIM (p < 0.001).
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In vivo magnetic resonance spectroscopy in the brain of Cdkl5 null mice reveals a metabolic profile indicative of mitochondrial dysfunctions
Sara Carli,Linda Chaabane,Clarissa Butti,Clara De Palma,Patrizia Aimar,Chiara Salio,Aglaia Vignoli,Maurizio Giustetto,Nicoletta Landsberger,Angelisa Frasca +9 more
TL;DR: In this paper, the authors used different Magnetic Resonance (MR) methods to disclose structural, functional, or metabolic signatures of Cdkl5 deficiency in the brain of adult mice.