Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins
about
Antamanide, a derivative of Amanita phalloides, is a novel inhibitor of the mitochondrial permeability transition poreHexokinase II detachment from mitochondria triggers apoptosis through the permeability transition pore independent of voltage-dependent anion channelsPhysical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient musclesInvestigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophyCyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathiesThe beneficial role of proteolysis in skeletal muscle growth and stress adaptationA TALEN-Exon Skipping Design for a Bethlem Myopathy Model in ZebrafishTargeting Mitochondria and Reactive Oxygen Species-Driven Pathogenesis in Diabetic NephropathyGenetic evidence in the mouse solidifies the calcium hypothesis of myofiber death in muscular dystrophyA mouse model for dominant collagen VI disorders: heterozygous deletion of Col6a3 Exon 16Apoptosis repressor with a CARD domain (ARC) restrains Bax-mediated pathogenesis in dystrophic skeletal muscleRecent advances using zebrafish animal models for muscle disease drug discoveryPhosphate is essential for inhibition of the mitochondrial permeability transition pore by cyclosporin A and by cyclophilin D ablationAdenine nucleotide translocase 1 deficiency results in dilated cardiomyopathy with defects in myocardial mechanics, histopathological alterations, and activation of apoptosis.Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophyMechanical and electrophysiological properties of the sarcolemma of muscle fibers in two murine models of muscle dystrophy: col6a1-/- and mdxAllele-specific Gene Silencing of Mutant mRNA Restores Cellular Function in Ullrich Congenital Muscular Dystrophy Fibroblasts.Col6a1 null mice as a model to study skin phenotypes in patients with collagen VI related myopathies: expression of classical and novel collagen VI variants during wound healing.The collagen VI-related myopathies Ullrich congenital muscular dystrophy and Bethlem myopathy.Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies.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.Role of the mitochondrion in programmed necrosisEarly-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice.Therapy of collagen VI-related myopathies (Bethlem and Ullrich).Gene expression profiling identifies molecular pathways associated with collagen VI deficiency and provides novel therapeutic targets.Defective collagen VI α6 chain expression in the skeletal muscle of patients with collagen VI-related myopathiesColVI myopathies: where do we stand, where do we go?Cyclosporine A in Ullrich congenital muscular dystrophy: long-term resultsMitochondrial energetics and therapeutics.Properties of Ca(2+) transport in mitochondria of Drosophila melanogasterBiomarkers in the assessment of therapies for familial amyloidotic polyneuropathy.Critical evaluation of the use of cell cultures for inclusion in clinical trials of patients affected by collagen VI myopathiesCyclophilin D, a target for counteracting skeletal muscle dysfunction in mitochondrial myopathyDiscovery, Synthesis, and Optimization of Diarylisoxazole-3-carboxamides as Potent Inhibitors of the Mitochondrial Permeability Transition Pore.Melanocytes--a novel tool to study mitochondrial dysfunction in Duchenne muscular dystrophy.Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy.Muscle LIM protein/CSRP3: a mechanosensor with a role in autophagy.Regulation and pharmacology of the mitochondrial permeability transition pore.Mitochondrial abnormalities, energy deficit and oxidative stress are features of calpain 3 deficiency in skeletal muscle
P2860
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P2860
Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@ast
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@en
type
label
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@ast
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@en
prefLabel
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@ast
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@en
P2093
P2860
P50
P356
P1476
Mitochondrial dysfunction in t ...... tive therapy with cyclosporins
@en
P2093
Alessandra Ferlini
Alessia Angelin
Cristina Golfieri
Francesca Gualandi
Nadir M Maraldi
Natascha Bergamin
Paolo Bonaldo
Paolo Grumati
Patrizia Sabatelli
Tania Tiepolo
P2860
P304
P356
10.1073/PNAS.0610270104
P407
P577
2007-01-10T00:00:00Z