Published in Am J Pathol on April 01, 2006
Dystrophins carrying spectrin-like repeats 16 and 17 anchor nNOS to the sarcolemma and enhance exercise performance in a mouse model of muscular dystrophy. J Clin Invest (2009) 2.89
Sildenafil and cardiomyocyte-specific cGMP signaling prevent cardiomyopathic changes associated with dystrophin deficiency. Proc Natl Acad Sci U S A (2008) 1.29
Nitrosative stress elicited by nNOSµ delocalization inhibits muscle force in dystrophin-null mice. J Pathol (2010) 1.20
Reciprocal amplification of ROS and Ca(2+) signals in stressed mdx dystrophic skeletal muscle fibers. Pflugers Arch (2009) 1.16
Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy. J Clin Invest (2012) 1.08
Subcutaneous injection, from birth, of epigallocatechin-3-gallate, a component of green tea, limits the onset of muscular dystrophy in mdx mice: a quantitative histological, immunohistochemical and electrophysiological study. Histochem Cell Biol (2008) 1.02
Uncoupling of increased cellular oxidative stress and myocardial ischemia reperfusion injury by directed sarcolemma stabilization. J Mol Cell Cardiol (2013) 0.95
Peroxisome proliferator-activated receptor γ coactivator1- gene α transfer restores mitochondrial biomass and improves mitochondrial calcium handling in post-necrotic mdx mouse skeletal muscle. J Physiol (2012) 0.92
Inflammatory monocytes promote progression of Duchenne muscular dystrophy and can be therapeutically targeted via CCR2. EMBO Mol Med (2014) 0.92
Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy. Physiol Rev (2016) 0.85
Inhibition of monocyte chemoattractant protein-1 prevents diaphragmatic inflammation and maintains contractile function during endotoxemia. Crit Care (2010) 0.80
The time course effects of electroacupuncture on promoting skeletal muscle regeneration and inhibiting excessive fibrosis after contusion in rabbits. Evid Based Complement Alternat Med (2013) 0.79
S-nitrosoglutathione reductase deficiency-induced S-nitrosylation results in neuromuscular dysfunction. Antioxid Redox Signal (2014) 0.79
Delayed cardiomyopathy in dystrophin deficient mdx mice relies on intrinsic glutathione resource. Am J Pathol (2010) 0.78
Improved Muscle Function in Duchenne Muscular Dystrophy through L-Arginine and Metformin: An Investigator-Initiated, Open-Label, Single-Center, Proof-Of-Concept-Study. PLoS One (2016) 0.77
Short-lived recombinant adeno-associated virus transgene expression in dystrophic muscle is associated with oxidative damage to transgene mRNA. Mol Ther Methods Clin Dev (2015) 0.76
Immunohistochemical and morphofunctional studies of skeletal muscle tissues with electric nerve stimulation by in vivo cryotechnique. Acta Histochem Cytochem (2015) 0.75
Skeletal muscle water T2 as a biomarker of disease status and exercise effects in patients with Duchenne muscular dystrophy. Neuromuscul Disord (2017) 0.75
Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell (1987) 20.27
Nitric oxide, superoxide, and peroxynitrite: the good, the bad, and ugly. Am J Physiol (1996) 13.14
Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc Natl Acad Sci U S A (1993) 7.18
The molecular basis of muscular dystrophy in the mdx mouse: a point mutation. Science (1989) 6.80
Interaction of nitric oxide synthase with the postsynaptic density protein PSD-95 and alpha1-syntrophin mediated by PDZ domains. Cell (1996) 6.42
Enzyme-independent formation of nitric oxide in biological tissues. Nat Med (1995) 4.28
Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy. Cell (1995) 4.21
Nanogram nitrite and nitrate determination in environmental and biological materials by vanadium (III) reduction with chemiluminescence detection. Anal Chem (1989) 3.92
Nitric oxide regulation of superoxide and peroxynitrite-dependent lipid peroxidation. Formation of novel nitrogen-containing oxidized lipid derivatives. J Biol Chem (1994) 3.64
A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. J Cell Biol (2001) 3.41
Animal models for muscular dystrophy show different patterns of sarcolemmal disruption. J Cell Biol (1997) 3.32
Biological tyrosine nitration: a pathophysiological function of nitric oxide and reactive oxygen species. Arch Biochem Biophys (1998) 3.21
Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature (1991) 3.19
Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle. Proc Natl Acad Sci U S A (1998) 3.00
Duchenne dystrophy: electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fiber. Neurology (1975) 2.89
Functional muscle ischemia in neuronal nitric oxide synthase-deficient skeletal muscle of children with Duchenne muscular dystrophy. Proc Natl Acad Sci U S A (2000) 2.49
Formation of reactive nitrogen species during peroxidase-catalyzed oxidation of nitrite. A potential additional mechanism of nitric oxide-dependent toxicity. J Biol Chem (1997) 2.48
7-Nitro indazole, an inhibitor of nitric oxide synthase, exhibits anti-nociceptive activity in the mouse without increasing blood pressure. Br J Pharmacol (1993) 1.97
A role for nitric oxide in muscle repair: nitric oxide-mediated activation of muscle satellite cells. Mol Biol Cell (2000) 1.75
Mechanical ventilation protects against diaphragm injury in sepsis: interaction of oxidative and mechanical stresses. Am J Respir Crit Care Med (2002) 1.70
Development of a fluorescent indicator for nitric oxide based on the fluorescein chromophore. Chem Pharm Bull (Tokyo) (1998) 1.60
Functional nitric oxide synthase mislocalization in cardiomyopathy. J Mol Cell Cardiol (2004) 1.58
Dystrophin-deficient cardiomyocytes are abnormally vulnerable to mechanical stress-induced contractile failure and injury. FASEB J (2001) 1.54
Myeloperoxidase and horseradish peroxidase catalyze tyrosine nitration in proteins from nitrite and hydrogen peroxide. Arch Biochem Biophys (1998) 1.54
Loss of dystrophin causes aberrant mechanotransduction in skeletal muscle fibers. FASEB J (2004) 1.50
Loss of cytoplasmic basic fibroblast growth factor from physiologically wounded myofibers of normal and dystrophic muscle. J Cell Sci (1993) 1.48
Dystrophin and the membrane skeleton. Curr Opin Cell Biol (1993) 1.47
Dystrobrevin and dystrophin: an interaction through coiled-coil motifs. Proc Natl Acad Sci U S A (1997) 1.45
Duchenne muscular dystrophy: plasma membrane loss initiates muscle cell necrosis unless it is repaired. Brain (1979) 1.44
Muscle fibers of mdx mice are more vulnerable to exercise than those of normal mice. Neuromuscul Disord (1997) 1.43
Purification of dystrophin from skeletal muscle. J Biol Chem (1991) 1.41
Evidence of oxidative stress in mdx mouse muscle: studies of the pre-necrotic state. J Neurol Sci (1998) 1.37
Muscle cells from mdx mice have an increased susceptibility to oxidative stress. Neuromuscul Disord (1998) 1.34
mdx muscle pathology is independent of nNOS perturbation. Hum Mol Genet (1998) 1.32
Exercise-stimulated glucose transport in skeletal muscle is nitric oxide dependent. Am J Physiol (1997) 1.28
Isoform diversity of dystrobrevin, the murine 87-kDa postsynaptic protein. J Biol Chem (1996) 1.27
Duchenne muscular dystrophy: functional ischemia reproduces its characteristic lesions. Science (1971) 1.26
Nitric oxide as a messenger molecule for myoblast fusion. J Biol Chem (1994) 1.24
Muscular dystrophy in mdx mice despite lack of neuronal nitric oxide synthase. J Neurochem (1998) 1.24
Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy. Hum Mol Genet (2000) 1.23
Transgenic mice expressing mutant caveolin-3 show severe myopathy associated with increased nNOS activity. Hum Mol Genet (2001) 1.23
Role of nitric oxide in skeletal muscle: synthesis, distribution and functional importance. Acta Physiol Scand (1998) 1.22
Role of nitric oxide in the pathogenesis of muscular dystrophies: a "two hit" hypothesis of the cause of muscle necrosis. Microsc Res Tech (2001) 1.21
Evidence of mdx mouse skeletal muscle fragility in vivo by eccentric running exercise. Muscle Nerve (1998) 1.18
Molecular pathophysiology of myofiber injury in deficiencies of the dystrophin-glycoprotein complex. Am J Phys Med Rehabil (2002) 1.17
Green tea extract decreases muscle necrosis in mdx mice and protects against reactive oxygen species. Am J Clin Nutr (2002) 1.15
Biophysical consequences of lipid peroxidation in membranes. Chem Phys Lipids (1987) 1.10
Nitric oxide protects the skeletal muscle Ca2+ release channel from oxidation induced activation. J Biol Chem (1997) 1.09
Protein-protein interactions controlling nitric oxide synthases. Acta Physiol Scand (2000) 1.01
Early onset of lipofuscin accumulation in dystrophin-deficient skeletal muscles of DMD patients and mdx mice. J Mol Histol (2004) 1.00
Skeletal muscle reperfusion injury is mediated by neutrophils and the complement membrane attack complex. Am J Physiol (1999) 0.99
Nitric oxide inhibits calpain-mediated proteolysis of talin in skeletal muscle cells. Am J Physiol Cell Physiol (2000) 0.98
Oxidative stress as a potential pathogenic mechanism in an animal model of Duchenne muscular dystrophy. Neuromuscul Disord (1997) 0.95
Nitric oxide modulates excitation-contraction coupling in the diaphragm. Comp Biochem Physiol A Mol Integr Physiol (1998) 0.94
Protein tyrosine nitration in the ventilatory muscles: role of nitric oxide synthases. Am J Respir Cell Mol Biol (2002) 0.90
Increased levels of serum-creatine-phosphokinase after transient limb ischaemia in patients with muscular dystrophy. Lancet (1970) 0.87
Role of leukocytes and tissue-derived oxidants in short-term skeletal muscle ischemia-reperfusion injury. Am J Physiol Heart Circ Physiol (2000) 0.86
Effects of iron deprivation on the pathology and stress protein expression in murine X-linked muscular dystrophy. Biochem Pharmacol (1998) 0.86
Peroxynitrite-induced hemolysis of human erythrocytes and its inhibition by antioxidants. FEBS Lett (1997) 0.85
Junctional sites of erythrocyte skeletal proteins are specific targets of tert-butylhydroperoxide oxidative damage. Chem Biol Interact (1995) 0.84
Dystrophin mutations predict cellular susceptibility to oxidative stress. Muscle Nerve (2000) 0.83
Free radical injury in skeletal muscle ischemia and reperfusion. J Surg Res (1991) 0.82
Indicators of oxidative injury and alterations of the cell membrane in the skeletal muscle of rats submitted to ischemia and reperfusion. J Surg Res (2000) 0.82
Differential protein oxidation in Duchenne and Becker muscular dystrophy. Neuroreport (1998) 0.82
Ischemia-induced structural change in SR Ca2+-ATPase is associated with reduced enzyme activity in rat muscle. Am J Physiol Regul Integr Comp Physiol (2001) 0.80
Endotoxin pretreatment inhibits neutrophil proliferation and function. J Surg Res (1994) 0.78
Nitric oxide synthase inhibition negates septic-induced alterations in cytoplasmic calcium homeostasis and membrane dynamics. Am Surg (1997) 0.78
Deferoxamine prevents lipid peroxidation and attenuates reoxygenation injury in postischemic skeletal muscle. Am J Physiol (1993) 0.78
Different roles of neuronal and endothelial nitric oxide synthases on ischemic nitric oxide production in gerbil striatum. Neurosci Lett (2000) 0.77
Effects of 4-h ischemia and 1-h reperfusion on rat muscle sarcoplasmic reticulum function. Am J Physiol Endocrinol Metab (2001) 0.77
Oxidative erythrocyte membrane damage in hereditary spherocytosis. Biochem Int (1992) 0.76
Ischemia/reperfusion-induced changes in intracellular free Ca2+ levels in rat skeletal muscle fibers--an in vivo study. Pflugers Arch (2000) 0.76
Rapidly progressive diaphragmatic weakness and injury during mechanical ventilation in humans. Am J Respir Crit Care Med (2010) 2.17
Controlled mechanical ventilation leads to remodeling of the rat diaphragm. Am J Respir Crit Care Med (2002) 1.83
Mechanical ventilation-induced diaphragm disuse in humans triggers autophagy. Am J Respir Crit Care Med (2010) 1.80
A role for endoglin in coupling eNOS activity and regulating vascular tone revealed in hereditary hemorrhagic telangiectasia. Circ Res (2005) 1.76
Upper airway muscle inflammation and denervation changes in obstructive sleep apnea. Am J Respir Crit Care Med (2004) 1.75
Mechanical ventilation protects against diaphragm injury in sepsis: interaction of oxidative and mechanical stresses. Am J Respir Crit Care Med (2002) 1.70
Preferential diaphragmatic weakness during sustained Pseudomonas aeruginosa lung infection. Am J Respir Crit Care Med (2003) 1.68
Ventilator-induced diaphragmatic dysfunction. Am J Respir Crit Care Med (2004) 1.66
Clinical review: ventilator-induced diaphragmatic dysfunction--human studies confirm animal model findings! Crit Care (2011) 1.55
Rapid onset of specific diaphragm weakness in a healthy murine model of ventilator-induced diaphragmatic dysfunction. Anesthesiology (2012) 1.50
Airway inflammation assessed by invasive and noninvasive means in severe asthma: eosinophilic and noneosinophilic phenotypes. J Allergy Clin Immunol (2006) 1.43
Mitochondrial dysfunction and lipid accumulation in the human diaphragm during mechanical ventilation. Am J Respir Crit Care Med (2012) 1.41
Six1 and Eya1 expression can reprogram adult muscle from the slow-twitch phenotype into the fast-twitch phenotype. Mol Cell Biol (2004) 1.39
Sleep-disordered breathing in fatigued postpoliomyelitis clinic patients. Arch Phys Med Rehabil (2006) 1.39
Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice. PLoS Genet (2009) 1.26
AMPK activation stimulates autophagy and ameliorates muscular dystrophy in the mdx mouse diaphragm. Am J Pathol (2012) 1.24
Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline. Can Respir J (2011) 1.17
Metabolic and signaling alterations in dystrophin-deficient hearts precede overt cardiomyopathy. J Mol Cell Cardiol (2007) 1.15
Bcl-2-associated autophagy regulator Naf-1 required for maintenance of skeletal muscle. Hum Mol Genet (2012) 1.14
CC family chemokines directly regulate myoblast responses to skeletal muscle injury. J Physiol (2008) 1.10
Profiling substrate fluxes in the isolated working mouse heart using 13C-labeled substrates: focusing on the origin and fate of pyruvate and citrate carbons. Am J Physiol Heart Circ Physiol (2003) 1.09
Toll-like receptors differentially regulate CC and CXC chemokines in skeletal muscle via NF-kappaB and calcineurin. Infect Immun (2006) 1.07
Long-term impact of bronchopulmonary dysplasia on pulmonary function. Can Respir J (2011) 1.05
Factors influencing the efficacy, longevity, and safety of electroporation-assisted plasmid-based gene transfer into mouse muscles. Mol Ther (2004) 1.05
Endotoxin triggers nuclear factor-kappaB-dependent up-regulation of multiple proinflammatory genes in the diaphragm. Am J Respir Crit Care Med (2006) 1.00
Alterations in mitochondrial function as a harbinger of cardiomyopathy: lessons from the dystrophic heart. J Mol Cell Cardiol (2009) 0.99
Sustained improvement of muscle function one year after full-length dystrophin gene transfer into mdx mice by a gutted helper-dependent adenoviral vector. Hum Gene Ther (2004) 0.98
Dimethylthiourea protects against chlorine induced changes in airway function in a murine model of irritant induced asthma. Respir Res (2010) 0.98
Optimization of regional intraarterial naked DNA-mediated transgene delivery to skeletal muscles in a large animal model. Mol Ther (2005) 0.93
Peroxisome proliferator-activated receptor γ coactivator1- gene α transfer restores mitochondrial biomass and improves mitochondrial calcium handling in post-necrotic mdx mouse skeletal muscle. J Physiol (2012) 0.92
Prolonged dystrophin expression and functional correction of mdx mouse muscle following gene transfer with a helper-dependent (gutted) adenovirus-encoding murine dystrophin. Hum Mol Genet (2003) 0.91
Dynamic responses of the glutathione system to acute oxidative stress in dystrophic mouse (mdx) muscles. Am J Physiol Regul Integr Comp Physiol (2006) 0.90
Airway delivery of soluble mycobacterial antigens restores protective mucosal immunity by single intramuscular plasmid DNA tuberculosis vaccination: role of proinflammatory signals in the lung. J Immunol (2008) 0.89
Factors associated with induced chronic inflammation in mdx skeletal muscle cause posttranslational stabilization and augmentation of extrasynaptic sarcolemmal utrophin. Hum Gene Ther (2005) 0.89
Impact of IL-10 on diaphragmatic cytokine expression and contractility during Pseudomonas Infection. Am J Respir Cell Mol Biol (2006) 0.88
Expression of dystrophin driven by the 1.35-kb MCK promoter ameliorates muscular dystrophy in fast, but not in slow muscles of transgenic mdx mice. Mol Ther (2003) 0.88
Expression and regulation of CC class chemokines in the dystrophic (mdx) diaphragm. Am J Respir Cell Mol Biol (2005) 0.88
Successful compensation for dystrophin deficiency by a helper-dependent adenovirus expressing full-length utrophin. Mol Ther (2007) 0.87
Stress-induced opening of the permeability transition pore in the dystrophin-deficient heart is attenuated by acute treatment with sildenafil. Am J Physiol Heart Circ Physiol (2010) 0.85
Targeting artificial transcription factors to the utrophin A promoter: effects on dystrophic pathology and muscle function. J Biol Chem (2008) 0.83
Regenerative capacity of the dystrophic (mdx) diaphragm after induced injury. Am J Physiol Regul Integr Comp Physiol (2004) 0.83
Therapeutic gene transfer to dystrophic diaphragm by an adenoviral vector deleted of all viral genes. Am J Physiol Lung Cell Mol Physiol (2004) 0.83
Affinity for MgADP and force of unbinding from actin of myosin purified from tonic and phasic smooth muscle. Am J Physiol Cell Physiol (2008) 0.82
Muscle-specific inhibition of the classical nuclear factor-κB pathway is protective against diaphragmatic weakness in murine endotoxemia. Crit Care Med (2014) 0.81
Simultaneous dystrophin and dysferlin deficiencies associated with high-level expression of the coxsackie and adenovirus receptor in transgenic mice. Am J Pathol (2006) 0.80
Inhibition of monocyte chemoattractant protein-1 prevents diaphragmatic inflammation and maintains contractile function during endotoxemia. Crit Care (2010) 0.80
Induction of nitric oxide synthase expression by lipopolysaccharide is mediated by calcium-dependent PKCα-β1 in alveolar epithelial cells. Am J Physiol Lung Cell Mol Physiol (2013) 0.80
Ultrasound increases plasmid-mediated gene transfer to dystrophic muscles without collateral damage. Mol Ther (2002) 0.78
Harvesting airway surface liquid: a comparison of two techniques. Pediatr Pulmonol (2004) 0.78
Diffusion capacity in children: what happens with exercise? Paediatr Respir Rev (2012) 0.78
Chemokine receptor and ligand upregulation in the diaphragm during endotoxemia and Pseudomonas lung infection. Mediators Inflamm (2009) 0.77
Diaphragmatic dysfunction in the intensive care unit: caught in the cross-fire between sepsis and mechanical ventilation. Crit Care (2013) 0.77
Nitric oxide production in the ventilatory muscles in response to acute resistive loading. Am J Physiol Lung Cell Mol Physiol (2006) 0.75
Molecular, cellular, and muscle strip mechanics of the mdx mouse diaphragm. Am J Physiol Cell Physiol (2013) 0.75
Response to letter. Pediatr Pulmonol (2014) 0.75
Relationship between Autophagy and Ventilator-induced Diaphragmatic Dysfunction. Anesthesiology (2015) 0.75
An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases. Ann Am Thorac Soc (2017) 0.75