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
Pharmacological activation of PPARβ/δ stimulates utrophin A expression in skeletal muscle fibers and restores sarcolemmal integrity in mature mdx mice
Pedro Miura,Joe V. Chakkalakal,Louise Boudreault,Guy Bélanger,Richard L. Hébert,Jean-Marc Renaud,Bernard J. Jasmin,Bernard J. Jasmin +7 more
TL;DR: Results demonstrate that pharmacological activation of PPARbeta/delta might provide functional benefits to DMD patients through enhancement of utrophin A expression.
Journal ArticleDOI
Epigenetic reprogramming of human embryonic stem cells into skeletal muscle cells and generation of contractile myospheres.
Sonia Albini,Paula Coutinho,Barbora Malecova,Lorenzo Giordani,Alex Savchenko,Sonia Vanina Forcales,Pier Lorenzo Puri +6 more
TL;DR: It is shown that selective absence of the SWI/SNF component BAF60C confers on hESCs resistance to MyoD-mediated activation of skeletal myogenesis, which establishes the molecular basis for the generation of hESC-derived myospheres exploitable for "disease in a dish" models of muscular physiology and dysfunction.
Journal ArticleDOI
Defects in mitochondrial ATP synthesis in dystrophin-deficient mdx skeletal muscles may be caused by complex I insufficiency
TL;DR: The data suggests that DMD pathology is exacerbated by a Complex I deficiency, which may contribute in part to the severe reductions in ATP production previously observed in dystrophic skeletal muscle.
Journal ArticleDOI
Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibition
TL;DR: Overall, data indicate that dystrophin-deficiency disrupts SSM localization, promotes mitochondrial inefficiency and restricts maximal mitochondrial ATP-generating capacity, which further understanding of how mitochondrial bioenergetic dysfunction contributes to disease pathogenesis in dystrophic skeletal muscle in vivo.
Journal ArticleDOI
Nitric oxide inhibition of Drp1-mediated mitochondrial fission is critical for myogenic differentiation
C. De Palma,Sestina Falcone,S. Pisoni,Sara Cipolat,Chris Panzeri,Sarah Pambianco,Addolorata Pisconti,Raffaele Allevi,Maria Teresa Bassi,Giulio Cossu,Tullio Pozzan,Salvador Moncada,Luca Scorrano,Silvia Brunelli,Emilio Clementi +14 more
TL;DR: It is shown that mitochondrial elongation is required for myogenesis to occur and that this event depends on the cellular generation of nitric oxide (NO), and that these effects of NO inhibition were not observed in myogenic precursor cells containing a dominant-negative form of Drp1.