Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy
about
Antamanide, a derivative of Amanita phalloides, is a novel inhibitor of the mitochondrial permeability transition poreRemodeling of Mitochondrial Flashes in Muscular Development and Dystrophy in ZebrafishGenetic evidence in the mouse solidifies the calcium hypothesis of myofiber death in muscular dystrophyAltered expression of cyclin A 1 in muscle of patients with facioscapulohumeral muscle dystrophy (FSHD-1)Gene-mediated restoration of normal myofiber elasticity in dystrophic muscles.Co-administration of ibuprofen and nitric oxide is an effective experimental therapy for muscular dystrophy, with immediate applicability to humans.Debio-025 is more effective than prednisone in reducing muscular pathology in mdx mice.Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies.Melanocytes from Patients Affected by Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy have Dysfunctional Mitochondria That Can be Rescued with Cyclophilin Inhibitors.Defects in mitochondrial ATP synthesis in dystrophin-deficient mdx skeletal muscles may be caused by complex I insufficiency.SERCA1 overexpression minimizes skeletal muscle damage in dystrophic mouse models.Mitochondrial dysfunctions during progression of dystrophic cardiomyopathyQuantitative evaluation of the beneficial effects in the mdx mouse of epigallocatechin gallate, an antioxidant polyphenol from green tea.Caloric restriction induces energy-sparing alterations in skeletal muscle contraction, fiber composition and local thyroid hormone metabolism that persist during catch-up fat upon refeeding.The Mitochondrial Permeability Transition Pore: Channel Formation by F-ATP Synthase, Integration in Signal Transduction, and Role in Pathophysiology.Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibitionMelanocytes--a novel tool to study mitochondrial dysfunction in Duchenne muscular dystrophy.The molecular composition of the mitochondrial permeability transition pore.Mitochondrial abnormalities, energy deficit and oxidative stress are features of calpain 3 deficiency in skeletal muscleAlterations in mitochondrial function as a harbinger of cardiomyopathy: lessons from the dystrophic heart.The physiological response of protease inhibition in dystrophic muscle.What has the mdx mouse model of Duchenne muscular dystrophy contributed to our understanding of this disease?Pharmacological therapeutics targeting the secondary defects and downstream pathology of Duchenne muscular dystrophyTreatment with human immunoglobulin G improves the early disease course in a mouse model of Duchenne muscular dystrophy.A Cell Culture System to Investigate the Presynaptic Control of Subsynaptic Membrane Differentiation at the Neuromuscular Junction.The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy.Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice.Chemical genetics unveils a key role of mitochondrial dynamics, cytochrome c release and IP3R activity in muscular dystrophy.Skeletal Muscle Metabolism in Duchenne and Becker Muscular Dystrophy-Implications for Therapies.FRET-Protease-Coupled Peptidyl-Prolyl cis-trans Isomerase AssayTamoxifen prolongs survival and alleviates symptoms in mice with fatal X-linked myotubular myopathy
P2860
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P2860
Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy
description
2008 nî lūn-bûn
@nan
2008 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@ast
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@en
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@nl
type
label
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@ast
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@en
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@nl
prefLabel
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@ast
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@en
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@nl
P2093
P2860
P356
P1476
Investigation of Debio 025, a ...... or Duchenne muscular dystrophy
@en
P2093
G Vuagniaux
J Reutenauer
O M Dorchies
O Patthey-Vuadens
P2860
P304
P356
10.1038/BJP.2008.285
P407
P50
P577
2008-10-01T00:00:00Z