Therapeutic strategies to address neuronal nitric oxide synthase deficiency and the loss of nitric oxide bioavailability in Duchenne Muscular Dystrophy.

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Therapeutic strategies to address neuronal nitric oxide synthase deficiency and the loss of nitric oxide bioavailability in Duchenne Muscular Dystrophy. is …
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review articleQ7318358
scholarly articleQ13442814

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P6179Dimensions Publication ID1085600609
P356DOI10.1186/S13023-017-0652-Y
P932PMC publication ID5445371
P698PubMed publication ID28545481

P50authorCara A TimpaniQ86409870
Emma RybalkaQ52152129
P2093author name stringAlan Hayes
P2860cites workDecreased total nitric oxide production in patients with duchenne muscular dystrophy.Q52553786
Nitric oxide activates or inhibits skeletal muscle ryanodine receptors depending on its concentration, membrane potential and ligand binding.Q53917676
Dietary nitrate supplementation reduces the O2 cost of low-intensity exercise and enhances tolerance to high-intensity exercise in humansQ56864000
Nitric oxide donor and non steroidal anti inflammatory drugs as a therapy for muscular dystrophies: Evidence from a safety study with pilot efficacy measures in adult dystrophic patientsQ59484926
Effect of sildenafil on skeletal and cardiac muscle in Becker muscular dystrophyQ60772304
Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophyQ24307833
Nitric oxide and peroxynitrite in health and diseaseQ24645400
Sildenafil and cardiomyocyte-specific cGMP signaling prevent cardiomyopathic changes associated with dystrophin deficiencyQ24655371
Nitric oxide synthases, S-nitrosylation and cardiovascular health: from molecular mechanisms to therapeutic opportunities (review)Q26823520
Skeletal muscle nitric oxide signaling and exercise: a focus on glucose metabolismQ26995484
Citrulline a more suitable substrate than arginine to restore NO production and the microcirculation during endotoxemiaQ27302422
Primary role of functional ischemia, quantitative evidence for the two-hit mechanism, and phosphodiesterase-5 inhibitor therapy in mouse muscular dystrophyQ27302822
The complete sequence of dystrophin predicts a rod-shaped cytoskeletal proteinQ28118471
Population frequencies of inherited neuromuscular diseases—A world surveyQ28266298
Mechanisms of nitrite bioactivationQ28384653
Functional and molecular effects of arginine butyrate and prednisone on muscle and heart in the mdx mouse model of Duchenne Muscular DystrophyQ28474542
Treatment with a nitric oxide-donating NSAID alleviates functional muscle ischemia in the mouse model of Duchenne muscular dystrophyQ28484851
Dystrophin: the protein product of the Duchenne muscular dystrophy locusQ29618077
Attempting to Compensate for Reduced Neuronal Nitric Oxide Synthase Protein with Nitrate Supplementation Cannot Overcome Metabolic Dysfunction but Rather Has Detrimental Effects in Dystrophin-Deficient mdx MuscleQ33586004
Long-term treatment with naproxcinod significantly improves skeletal and cardiac disease phenotype in the mdx mouse model of dystrophyQ33649312
Drug screening in a zebrafish model of Duchenne muscular dystrophy.Q33845739
Regulation of cAMP and cGMP signaling: new phosphodiesterases and new functionsQ33860484
The clinical pharmacology of L-arginineQ33939368
PDE5 inhibition alleviates functional muscle ischemia in boys with Duchenne muscular dystrophyQ33987140
Neuronal nitric oxide synthase-rescue of dystrophin/utrophin double knockout mice does not require nNOS localization to the cell membraneQ34049251
Dietary inorganic nitrate reverses features of metabolic syndrome in endothelial nitric oxide synthase-deficient miceQ34199764
Sildenafil reverses cardiac dysfunction in the mdx mouse model of Duchenne muscular dystrophy.Q34279254
Sildenafil does not improve cardiomyopathy in Duchenne/Becker muscular dystrophy.Q34315276
Evaluation of bacterial nitrate reduction in the human oral cavityQ34391222
Dietary nitrate increases arginine availability and protects mitochondrial complex I and energetics in the hypoxic rat heart.Q34619501
Potential oxyradical damage and energy status in individual muscle fibres from degenerating muscle diseasesQ68045270
Effect of Duchenne muscular dystrophy on enzymes of energy metabolism in individual muscle fibersQ69075882
EMG computerized analysis of localized fatigue in Duchenne muscular dystrophyQ70197822
Decreased insulin receptors but normal glucose metabolism in Duchenne muscular dystrophyQ70298216
Immunohistochemical study of calpain and its endogenous inhibitor in the skeletal muscle of muscular dystrophyQ71905306
The serum nitric oxide levels in patients with Duchenne muscular dystrophyQ73801088
Tissue distribution of phosphodiesterase families and the effects of sildenafil on tissue cyclic nucleotides, platelet function, and the contractile responses of trabeculae carneae and aortic rings in vitroQ74607760
The effect of age and temperature on mdx muscle fatigueQ74759953
Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilationQ78616932
Cyclic nucleotide phosphodiesterase isozymes expressed in mouse skeletal muscleQ78878396
Dietary nitrate supplementation reduces the O2 cost of walking and running: a placebo-controlled studyQ82431001
Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complexQ82995100
Acute dietary nitrate supplementation improves cycling time trial performanceQ83799502
Dietary nitrate supplementation improves rowing performance in well-trained rowersQ84402341
No improvement in endurance performance after a single dose of beetroot juiceQ84581034
The effect of nitrate supplementation on muscle contraction in healthy adultsQ86935044
Nitrate supplementation enhances the contractile properties of human skeletal muscleQ87556092
Effect of dietary nitrate supplementation on tolerance to supramaximal intensity intermittent exerciseQ50444009
Beetroot juice supplementation reduces whole body oxygen consumption but does not improve indices of mitochondrial efficiency in human skeletal muscle.Q51584583
Evidence for a myogenic stem cell that is exhausted in dystrophic muscle.Q52168124
Does utrophin expression in muscles of mdx mice during postnatal development functionally compensate for dystrophin deficiency?Q52217515
Interactions between bioenergetics and mitochondrial biogenesisQ41015290
Sildenafil reduces respiratory muscle weakness and fibrosis in the mdx mouse model of Duchenne muscular dystrophyQ41031903
Nuclear recruitment of neuronal nitric-oxide synthase by α-syntrophin is crucial for the induction of mitochondrial biogenesisQ41848388
Cardiac phosphodiesterase 5 (cGMP-specific) modulates beta-adrenergic signaling in vivo and is down-regulated in heart failureQ42507993
Dietary nitrate increases tetanic [Ca2+]i and contractile force in mouse fast-twitch muscleQ42717596
Ibuprofen plus isosorbide dinitrate treatment in the mdx mice ameliorates dystrophic heart structure.Q43697562
Creatine supplementation reduces skeletal muscle degeneration and enhances mitochondrial function in mdx miceQ43818941
A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin.Q43860533
Expression of a NOS transgene in dystrophin-deficient muscle reduces muscle membrane damage without increasing the expression of membrane-associated cytoskeletal proteinsQ44807148
Defects in neuromuscular junction structure in dystrophic muscle are corrected by expression of a NOS transgene in dystrophin-deficient muscles, but not in muscles lacking alpha- and beta1-syntrophinsQ44967389
Nitrate supplementation's improvement of 10-km time-trial performance in trained cyclistsQ44988842
Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblastsQ45242416
Neuronal nitric oxide synthase isoforms alpha and mu are closely related calpain-sensitive proteins.Q45937201
Sodium nitrate alleviates functional muscle ischaemia in patients with Becker muscular dystrophyQ46088505
Cardiomyopathy in dystrophin-deficient hearts is prevented by expression of a neuronal nitric oxide synthase transgene in the myocardiumQ46285519
Expression of a muscle-specific, nitric oxide synthase transgene prevents muscle membrane injury and reduces muscle inflammation during modified muscle use in miceQ46485498
Systemic administration of L-arginine benefits mdx skeletal muscle functionQ46664538
L-arginine improves dystrophic phenotype in mdx miceQ46682624
Persistent and improved functional gain in mdx dystrophic mice after treatment with L-arginine and deflazacortQ46933224
Increased caveolin-3 levels in mdx mouse musclesQ47763919
A nitrate ester of sedative alkyl alcohol improves muscle function and structure in a murine model of Duchenne muscular dystrophyQ47836273
Impaired mitochondrial oxidative phosphorylation in skeletal muscle of the dystrophin-deficient mdx mouseQ47878704
Revisiting the dystrophin-ATP connection: How half a century of research still implicates mitochondrial dysfunction in Duchenne Muscular Dystrophy aetiologyQ47966217
Dietary nitrate supplementation in COPD: an acute, double-blind, randomized, placebo-controlled, crossover trialQ48005929
Arginine butyrate: a therapeutic candidate for Duchenne muscular dystrophy.Q48153388
Nitric oxide donors improve prednisone effects on muscular dystrophy in the mdx mouse diaphragmQ50283542
Dietary inorganic nitrate improves mitochondrial efficiency in humans.Q34626699
Defects in mitochondrial ATP synthesis in dystrophin-deficient mdx skeletal muscles may be caused by complex I insufficiency.Q34776381
Food sources of nitrates and nitrites: the physiologic context for potential health benefitsQ34980790
Dystrophic muscle improvement in zebrafish via increased heme oxygenase signaling.Q35043351
Dietary nitrate supplementation enhances exercise performance in peripheral arterial diseaseQ35057110
Nitric oxide signalling pathway in Duchenne muscular dystrophy mice: up-regulation of L-arginine transportersQ35195095
Dietary nitrate supplementation improves exercise performance and decreases blood pressure in COPD patientsQ35542901
Nitric oxide release combined with nonsteroidal antiinflammatory activity prevents muscular dystrophy pathology and enhances stem cell therapyQ35575973
Novel mini-dystrophin gene dual adeno-associated virus vectors restore neuronal nitric oxide synthase expression at the sarcolemmaQ35678425
Nitrate enhances skeletal muscle fatty acid oxidation via a nitric oxide-cGMP-PPAR-mediated mechanismQ35875662
Improved Muscle Function in Duchenne Muscular Dystrophy through L-Arginine and Metformin: An Investigator-Initiated, Open-Label, Single-Center, Proof-Of-Concept-Study.Q35901453
Naproxcinod shows significant advantages over naproxen in the mdx model of Duchenne Muscular DystrophyQ35986842
Discovery of Metabolic Biomarkers for Duchenne Muscular Dystrophy within a Natural History StudyQ35991086
A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx miceQ36294012
Deficient ryanodine receptor S-nitrosylation increases sarcoplasmic reticulum calcium leak and arrhythmogenesis in cardiomyocytesQ36300139
Sarcolemmal targeting of nNOSμ improves contractile function of mdx muscleQ36402599
Detection of deletions spanning the Duchenne muscular dystrophy locus using a tightly linked DNA segmentQ36423793
Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibitionQ36467072
L-arginine decreases inflammation and modulates the nuclear factor-kappaB/matrix metalloproteinase cascade in mdx muscle fibersQ36691055
Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscleQ36760419
Sarcolemma-localized nNOS is required to maintain activity after mild exerciseQ36987100
The nitrate-nitrite-nitric oxide pathway in physiology and therapeuticsQ37048314
Dystrophins carrying spectrin-like repeats 16 and 17 anchor nNOS to the sarcolemma and enhance exercise performance in a mouse model of muscular dystrophy.Q37111368
Dual AAV therapy ameliorates exercise-induced muscle injury and functional ischemia in murine models of Duchenne muscular dystrophyQ37111962
Treatment with L-citrulline and metformin in Duchenne muscular dystrophy: study protocol for a single-centre, randomised, placebo-controlled trialQ37150891
Tadalafil Treatment Delays the Onset of Cardiomyopathy in Dystrophin-Deficient HeartsQ37240478
Loss of positive allosteric interactions between neuronal nitric oxide synthase and phosphofructokinase contributes to defects in glycolysis and increased fatigability in muscular dystrophyQ37312499
Neuronal nitric oxide synthase and dystrophin-deficient muscular dystrophyQ37387484
Cardiorespiratory function associated with dietary nitrate supplementationQ37541277
Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy.Q37604365
New Pharmacological Strategies to Increase cGMP.Q38608909
Low doses of arginine butyrate derivatives improve dystrophic phenotype and restore membrane integrity in DMD models.Q39017870
A biochemical study of certain skeletal muscle constituents in human progressive muscular dystrophyQ40330875
Nitrate, nitrite and N-nitroso compounds.Q40600062
Pathways Implicated in Tadalafil Amelioration of Duchenne Muscular Dystrophy.Q40813682
Nitric oxide and l-arginine cause an accumulation of utrophin at the sarcolemma: a possible compensation for dystrophin loss in Duchenne muscular dystrophyQ40910686
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P433issue1
P407language of work or nameEnglishQ1860
P921main subjectbioavailabilityQ461809
Duchenne muscular dystrophyQ1648484
mechanism of actionQ3271540
enzymeQ8047
P5008on focus list of Wikimedia projectScienceSourceQ55439927
P304page(s)100
P577publication date2017-05-25
P1433published inOrphanet Journal of Rare DiseasesQ15756117
P1476titleTherapeutic strategies to address neuronal nitric oxide synthase deficiency and the loss of nitric oxide bioavailability in Duchenne Muscular Dystrophy
P478volume12

Reverse relations

cites work (P2860)
Q52564618Combined Therapies for Duchenne Muscular Dystrophy to Optimize Treatment Efficacy.
Q91811820Metabolic Alterations in Cardiomyocytes of Patients with Duchenne and Becker Muscular Dystrophies
Q55515149Skeletal Muscle Metabolism in Duchenne and Becker Muscular Dystrophy-Implications for Therapies.
Q92637631The Role of Macrophage Migration Inhibitory Factor in Alzheimer's Disease: Conventionally Pathogenetic or Unconventionally Protective?

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