Published in J Pharmacol Exp Ther on January 01, 2003
Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy. Neurobiol Dis (2008) 2.20
Preclinical drug trials in the mdx mouse: assessment of reliable and sensitive outcome measures. Muscle Nerve (2009) 2.19
Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse. PLoS One (2011) 1.29
Duchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies. Int J Exp Pathol (2003) 1.20
Effects of prednisolone on skeletal muscle contractility in mdx mice. Muscle Nerve (2009) 1.14
A multidisciplinary evaluation of the effectiveness of cyclosporine a in dystrophic mdx mice. Am J Pathol (2005) 1.10
Functional and molecular effects of arginine butyrate and prednisone on muscle and heart in the mdx mouse model of Duchenne Muscular Dystrophy. PLoS One (2010) 1.09
Inhibitory control over Ca(2+) sparks via mechanosensitive channels is disrupted in dystrophin deficient muscle but restored by mini-dystrophin expression. PLoS One (2008) 1.08
Microarchitecture is severely compromised but motor protein function is preserved in dystrophic mdx skeletal muscle. Biophys J (2010) 1.08
Importance of functional and metabolic impairments in the characterization of the C-26 murine model of cancer cachexia. Dis Model Mech (2012) 1.08
The role of fibrosis in Duchenne muscular dystrophy. Acta Myol (2012) 1.04
Understanding the process of fibrosis in Duchenne muscular dystrophy. Biomed Res Int (2014) 1.00
Enalapril treatment discloses an early role of angiotensin II in inflammation- and oxidative stress-related muscle damage in dystrophic mdx mice. Pharmacol Res (2011) 0.96
Niflumic acid inhibits chloride conductance of rat skeletal muscle by directly inhibiting the CLC-1 channel and by increasing intracellular calcium. Br J Pharmacol (2006) 0.96
Pre-clinical drug tests in the mdx mouse as a model of dystrophinopathies: an overview. Acta Myol (2012) 0.94
Bcl-2 overexpression prevents calcium overload and subsequent apoptosis in dystrophic myotubes. Biochem J (2006) 0.94
Evaluation of skeletal and cardiac muscle function after chronic administration of thymosin beta-4 in the dystrophin deficient mouse. PLoS One (2010) 0.90
Novel and optimized strategies for inducing fibrosis in vivo: focus on Duchenne Muscular Dystrophy. Skelet Muscle (2014) 0.89
Eccentric exercise in aging and diseased skeletal muscle: good or bad? J Appl Physiol (1985) (2013) 0.89
Influence of methylene blue on microglia-induced inflammation and motor neuron degeneration in the SOD1(G93A) model for ALS. PLoS One (2012) 0.89
Therapeutic Approaches to Genetic Ion Channelopathies and Perspectives in Drug Discovery. Front Pharmacol (2016) 0.86
Taurine: the appeal of a safe amino acid for skeletal muscle disorders. J Transl Med (2015) 0.85
Functional and Morphological Improvement of Dystrophic Muscle by Interleukin 6 Receptor Blockade. EBioMedicine (2015) 0.84
β1-syntrophin modulation by miR-222 in mdx mice. PLoS One (2010) 0.83
Paracrine effects of IGF-1 overexpression on the functional decline due to skeletal muscle disuse: molecular and functional evaluation in hindlimb unloaded MLC/mIgf-1 transgenic mice. PLoS One (2013) 0.82
Prednisolone treatment and restricted physical activity further compromise bone of mdx mice. J Musculoskelet Neuronal Interact (2012) 0.82
Myogenic differentiation induces taurine transporter in association with taurine-mediated cytoprotection in skeletal muscles. Biochem J (2006) 0.82
The physiological effects of IGF-1 (class 1:Ea transgene) over-expression on exercise-induced damage and adaptation in dystrophic muscles of mdx mice. Pflugers Arch (2008) 0.81
Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons. J Cell Sci (2010) 0.80
Increasing taurine intake and taurine synthesis improves skeletal muscle function in the mdx mouse model for Duchenne muscular dystrophy. J Physiol (2016) 0.79
Levels of inflammation and oxidative stress, and a role for taurine in dystropathology of the Golden Retriever Muscular Dystrophy dog model for Duchenne Muscular Dystrophy. Redox Biol (2016) 0.78
Nutrition strategies to improve physical capabilities in Duchenne muscular dystrophy. Phys Med Rehabil Clin N Am (2011) 0.78
Activation of osmolyte pathways in inflammatory myopathy and Duchenne muscular dystrophy points to osmoregulation as a contributing pathogenic mechanism. Lab Invest (2016) 0.78
The different impact of a high fat diet on dystrophic mdx and control C57Bl/10 mice. PLoS Curr (2011) 0.77
Age-dependent chloride channel expression in skeletal muscle fibres of normal and HSA(LR) myotonic mice. J Physiol (2012) 0.77
Comparison of Experimental Protocols of Physical Exercise for mdx Mice and Duchenne Muscular Dystrophy Patients. J Neuromuscul Dis (2015) 0.77
Angiotensin II modulates mouse skeletal muscle resting conductance to chloride and potassium ions and calcium homeostasis via the AT1 receptor and NADPH oxidase. Am J Physiol Cell Physiol (2014) 0.77
Increased levels of interleukin-6 exacerbate the dystrophic phenotype in mdx mice. Hum Mol Genet (2015) 0.77
Metabogenic and Nutriceutical Approaches to Address Energy Dysregulation and Skeletal Muscle Wasting in Duchenne Muscular Dystrophy. Nutrients (2015) 0.77
Increased taurine in pre-weaned juvenile mdx mice greatly reduces the acute onset of myofibre necrosis and dystropathology and prevents inflammation. PLoS Curr (2016) 0.76
Bmi1 enhances skeletal muscle regeneration through MT1-mediated oxidative stress protection in a mouse model of dystrophinopathy. J Exp Med (2014) 0.76
Novel compounds for the treatment of Duchenne muscular dystrophy: emerging therapeutic agents. Appl Clin Genet (2011) 0.75
Muscle damage, metabolism, and oxidative stress in mdx mice: Impact of aerobic running. Muscle Nerve (2016) 0.75
Naproxcinod shows significant advantages over naproxen in the mdx model of Duchenne Muscular Dystrophy. Orphanet J Rare Dis (2015) 0.75
Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle. Skelet Muscle (2016) 0.75
Risk of Myopathy in Patients in Therapy with Statins: Identification of Biological Markers in a Pilot Study. Front Pharmacol (2017) 0.75
Constitutive activation of MAPK cascade in acute quadriplegic myopathy. Ann Neurol (2004) 1.77
Phenotypic heterogeneity of the 8344A>G mtDNA "MERRF" mutation. Neurology (2013) 1.55
Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. Am J Hum Genet (2009) 1.47
Therapeutic approaches to ion channel diseases. Adv Genet (2008) 1.32
The gene for paroxysmal non-kinesigenic dyskinesia encodes an enzyme in a stress response pathway. Hum Mol Genet (2004) 1.29
Nonsense-mediated mRNA decay and ubiquitin-proteasome system regulate cardiac myosin-binding protein C mutant levels in cardiomyopathic mice. Circ Res (2009) 1.26
Redox homeostasis, oxidative stress and disuse muscle atrophy. J Physiol (2011) 1.25
Novel GNE mutations in Italian families with autosomal recessive hereditary inclusion-body myopathy. Hum Mutat (2004) 1.14
Spinal muscular atrophy associated with progressive myoclonic epilepsy is caused by mutations in ASAH1. Am J Hum Genet (2012) 1.11
Hybrid assemblies of ATP-sensitive K+ channels determine their muscle-type-dependent biophysical and pharmacological properties. Proc Natl Acad Sci U S A (2006) 1.11
A multidisciplinary evaluation of the effectiveness of cyclosporine a in dystrophic mdx mice. Am J Pathol (2005) 1.10
Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy. Brain (2009) 1.10
The m.3243A>G mitochondrial DNA mutation and related phenotypes. A matter of gender? J Neurol (2013) 1.09
Molecular determinants of differential pore blocking of kidney CLC-K chloride channels. EMBO Rep (2004) 1.09
P525L FUS mutation is consistently associated with a severe form of juvenile amyotrophic lateral sclerosis. Neuromuscul Disord (2011) 1.08
Acute auditory agnosia as the presenting hearing disorder in MELAS. Neurol Sci (2008) 1.08
Recovery of the soleus muscle after short- and long-term disuse induced by hindlimb unloading: effects on the electrical properties and myosin heavy chain profile. Neurobiol Dis (2005) 1.06
Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse. Brain (2002) 1.06
Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles. Pharmacol Res (2010) 1.05
Gentamicin treatment in exercised mdx mice: Identification of dystrophin-sensitive pathways and evaluation of efficacy in work-loaded dystrophic muscle. Neurobiol Dis (2008) 1.03
Microgravity during spaceflight directly affects in vitro osteoclastogenesis and bone resorption. FASEB J (2009) 1.02
Decrease in resting calcium and calcium entry associated with slow-to-fast transition in unloaded rat soleus muscle. FASEB J (2003) 1.02
Disuse of rat muscle in vivo reduces protein kinase C activity controlling the sarcolemma chloride conductance. J Physiol (2007) 1.01
Molecular requisites for drug binding to muscle CLC-1 and renal CLC-K channel revealed by the use of phenoxy-alkyl derivatives of 2-(p-chlorophenoxy)propionic acid. Mol Pharmacol (2002) 1.01
Phenotype-dependent functional and pharmacological properties of BK channels in skeletal muscle: effects of microgravity. Neurobiol Dis (2005) 1.00
Multiple pathological events in exercised dystrophic mdx mice are targeted by pentoxifylline: outcome of a large array of in vivo and ex vivo tests. J Appl Physiol (1985) (2009) 1.00
Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb-unloaded mice. Exp Physiol (2009) 0.99
Investigations of pharmacologic properties of the renal CLC-K1 chloride channel co-expressed with barttin by the use of 2-(p-Chlorophenoxy)propionic acid derivatives and other structurally unrelated chloride channels blockers. J Am Soc Nephrol (2004) 0.99
Response to therapy in myasthenia gravis with anti-MuSK antibodies. Ann N Y Acad Sci (2008) 0.99
Fluvastatin and atorvastatin affect calcium homeostasis of rat skeletal muscle fibers in vivo and in vitro by impairing the sarcoplasmic reticulum/mitochondria Ca2+-release system. J Pharmacol Exp Ther (2007) 0.98
Redefining phenotypes associated with mitochondrial DNA single deletion. J Neurol (2015) 0.98
Mitochondrial encephalomyopathies:gene mutation. Neuromuscul Disord (2003) 0.95
Mechanisms of block of muscle type CLC chloride channels (Review). Mol Membr Biol (2003) 0.95
Mitochondrial encephalomyopathies: gene mutation. Neuromuscul Disord (2004) 0.95
Fiber type-related changes in rat skeletal muscle calcium homeostasis during aging and restoration by growth hormone. Neurobiol Dis (2005) 0.94
Different ability of clenbuterol and salbutamol to block sodium channels predicts their therapeutic use in muscle excitability disorders. Mol Pharmacol (2003) 0.93
First evaluation of the potential effectiveness in muscular dystrophy of a novel chimeric compound, BN 82270, acting as calpain-inhibitor and anti-oxidant. Neuromuscul Disord (2006) 0.92
The alteration of calcium homeostasis in adult dystrophic mdx muscle fibers is worsened by a chronic exercise in vivo. Neurobiol Dis (2004) 0.92
New potent mexiletine and tocainide analogues evaluated in vivo and in vitro as antimyotonic agents on the myotonic ADR mouse. Neuromuscul Disord (2004) 0.92
Reduced expression of Kir6.2/SUR2A subunits explains KATP deficiency in K+-depleted rats. Neuromuscul Disord (2007) 0.90
Pre-clinical trials in Duchenne dystrophy: what animal models can tell us about potential drug effectiveness. Neuromuscul Disord (2002) 0.90
Mechanism of interaction of niflumic acid with heterologously expressed kidney CLC-K chloride channels. J Membr Biol (2007) 0.89
Taurine and skeletal muscle disorders. Neurochem Res (2004) 0.89
Splicing of the rSlo gene affects the molecular composition and drug response of Ca2+-activated K+ channels in skeletal muscle. PLoS One (2012) 0.88
On the metabolically active form of metaglidasen: improved synthesis and investigation of its peculiar activity on peroxisome proliferator-activated receptors and skeletal muscles. ChemMedChem (2015) 0.86
Overactivity of exercise-sensitive cation channels and their impaired modulation by IGF-1 in mdx native muscle fibers: beneficial effect of pentoxifylline. Neurobiol Dis (2006) 0.86
Functional characterization of ClC-1 mutations from patients affected by recessive myotonia congenita presenting with different clinical phenotypes. Exp Neurol (2013) 0.86
Involvement of 3Na+/2K+ ATP-ase and Pi-3 kinase in the response of skeletal muscle ATP-sensitive K+ channels to insulin. Neuromuscul Disord (2003) 0.85
Rippling muscle disease and cardiomyopathy associated with a mutation in the CAV3 gene. Neuromuscul Disord (2009) 0.85
Nerve growth factor eye drop administration improves visual function in a patient with optic glioma. Neurorehabil Neural Repair (2011) 0.85
Copper deficiency myelopathy: A report of two cases. J Spinal Cord Med (2014) 0.85
N-aryl-2,6-dimethylbenzamides, a new generation of tocainide analogues as blockers of skeletal muscle voltage-gated sodium channels. J Med Chem (2014) 0.84
Emerging role of calcium-activated potassium channel in the regulation of cell viability following potassium ions challenge in HEK293 cells and pharmacological modulation. PLoS One (2013) 0.84
Potential benefits of taurine in the prevention of skeletal muscle impairment induced by disuse in the hindlimb-unloaded rat. Amino Acids (2011) 0.84
Carbonic anhydrase inhibitors ameliorate the symptoms of hypokalaemic periodic paralysis in rats by opening the muscular Ca2+-activated-K+ channels. Neuromuscul Disord (2005) 0.84
Spontaneous recovery from anti-NMDAR encephalitis. J Neurol (2012) 0.83
Growth hormone secretagogues modulate the electrical and contractile properties of rat skeletal muscle through a ghrelin-specific receptor. Br J Pharmacol (2003) 0.83
Insulin-like growth factor I in inclusion-body myositis and human muscle cultures. J Neuropathol Exp Neurol (2004) 0.83
Paracrine effects of IGF-1 overexpression on the functional decline due to skeletal muscle disuse: molecular and functional evaluation in hindlimb unloaded MLC/mIgf-1 transgenic mice. PLoS One (2013) 0.82
Myoclonus in mitochondrial disorders. Mov Disord (2014) 0.82
An olive oil-derived antioxidant mixture ameliorates the age-related decline of skeletal muscle function. Age (Dordr) (2013) 0.82
Structural requisites of 2-(p-chlorophenoxy)propionic acid analogues for activity on native rat skeletal muscle chloride conductance and on heterologously expressed CLC-1. Br J Pharmacol (2003) 0.82
Clinical, Molecular, and Functional Characterization of CLCN1 Mutations in Three Families with Recessive Myotonia Congenita. Neuromolecular Med (2015) 0.81
"Myo-cardiomyopathy" is commonly associated with the A8344G "MERRF" mutation. J Neurol (2015) 0.81
Genotype-phenotype correlation in Pompe disease, a step forward. Orphanet J Rare Dis (2014) 0.81
Enzyme replacement therapy improves respiratory outcomes in patients with late-onset type II glycogenosis and high ventilator dependency. Lung (2013) 0.81
Assessment of a symptomatic Duchenne muscular dystrophy carrier 20 years after myoblast transplantation from her asymptomatic identical twin sister. Neuromuscul Disord (2013) 0.81
Stereospecific synthesis of "para-hydroxymexiletine" and sodium channel blocking activity evaluation. Chirality (2004) 0.81
New motor outcome function measures in evaluation of late-onset Pompe disease before and after enzyme replacement therapy. Muscle Nerve (2012) 0.80
In vivo evaluation of antimyotonic efficacy of β-adrenergic drugs in a rat model of myotonia. Neuropharmacology (2012) 0.79
Sleep disordered breathing in a cohort of patients with sporadic inclusion body myositis. Clin Neurophysiol (2013) 0.78
Pathogenic role of mtDNA duplications in mitochondrial diseases associated with mtDNA deletions. Am J Med Genet A (2003) 0.78
Hydroxylated analogs of mexiletine as tools for structural-requirements investigation of the sodium channel blocking activity. Arch Pharm (Weinheim) (2010) 0.78