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Welcome to the Pubget Medical Subject Headings (MESH) browser. Click on a topic or subtopic below to explore related papers. In the gold box is the most recent paper about the current MESH term, and below are 20 related papers.
Muscular Dystrophy, Animal (1):
Recent article
Muscular Dystrophy, Animal
Abstract
We show that the extracellular matrix protein biglycan regulates utrophin expression in immature muscle and that recombinant human biglycan (rhBGN) increases utrophin expression in cultured myotubes. Systemically delivered rhBGN up-regulates utrophin at the sarcolemma and reduces muscle pathology in...
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PMID: 21187385
PDF is available here.
PDF is available here.
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Abstract
We investigated systemic gene delivery in 4-day-old GRMD dogs given a single intravenous injection of an AAV9 vector (1.5 x 10(14) vector genomes/kg) carrying a human codon-optimized human mini-dystrophin gene under control of the cytomegalovirus (CMV) promoter. One of the three treated dogs was eut...
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PMID: 20517298
PDF is available here.
PDF is available here.
Ultrasonics (50)8 2010
Abstract
We recorded an increase in ultrasonic wave attenuation in the dystrophin-deficient samples in comparison with normal muscles. A correlation between attenuation, mouse age and the percentage of non-muscular proportion in muscle was observed. As Mdx mouse is the best animal model for DMD and reproduce...
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PMID: 20462623
PDF is available here.
PDF is available here.
Experimental Physiology (95)5 2010
Abstract
Experimental Physiology (95)5 2010
Abstract
I present a two-stage hypothesis for muscle damage in the dystrophinopathies, as follows: stage 1, the absence of dystrophin disrupts ion channel function, causing an activation of necrotizing Ca(2+)-activated proteases, which results in regenerated branched fibres; and stage 2, branched fibres are...
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PMID: 20139167
PDF is available here.
PDF is available here.
Molecular and Cellular Biochemistry (337)1-2 2010
Abstract
These results support the hypothesis that p53 plays some role in TNF-mediated necrosis of dystrophic muscle and present a potential new target for therapeutic interventions....
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PMID: 19859789
PDF is available here.
PDF is available here.
Canadian Journal of Physiology and Pharmacology/Revue Canadienne de Physiologie et Pharmacologie (88)2 2010
Abstract
We hypothesized that hypochlorous acid (HOCl), a strong H2O2-related free radical, damages contractile proteins and the sarcoplasmic reticulum. The aim of the present study was to investigate the effects of exposure to oxidative stress, generated by applying HOCl (100 micromol/L and 1 mmol/L), on th...
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PMID: 20237587
PDF is available here.
PDF is available here.
Abstract
I clinical trials in man. There is no longer any doubt that antisense oligomers can redirect dystrophin gene processing and by-pass protein truncating mutations after direct injection into muscle. Proof-of-concept has been demonstrated in human dystrophic muscle, with trials in Leiden and London sho...
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PMID: 20041827
PDF is available here.
PDF is available here.
Muscle development and regeneration in normal and pathological conditions: learning from Drosophila.
Abstract
We provide a current view on genetic mechanisms underlying muscle regeneration with a special focus on regeneration processes that take place in diseased and aging human muscle. Through examples of Drosophila models of human muscular diseases, we discuss potential impact they might have on uncoverin...
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PMID: 20041821
PDF is available here.
PDF is available here.
Abstract
We report that most of the proteins of the DGC are actually present at normal levels in the mdx mouse muscle plasma membrane. The proteins are detected in dystrophic animal muscles when the immunoblot assay is performed with crude surface membrane fractions instead of the usually employed KCl-washed...
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PMID: 19997781
PDF is available here.
PDF is available here.
Abstract
Duchenne muscular dystrophy (DMD), which results from deficiency in dystrophin, a sarcolemma protein of skeletal, cardiac and smooth muscle, is characterized by progressive striated muscle degeneration, but various gastrointestinal clinical manifestations have been observed. The aim was to evaluate...
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PMID: 19784719
PDF is available here.
PDF is available here.
Cell Transplantation (19)5 2010
Abstract
We investigated the effects of myoblasts injected with cardiotoxin on the contractile properties and resistance to eccentric contractions of transplanted and nontransplanted muscles. We used the extensor digitorum longus (EDL) as a model for our study. We conclude that the sole presence of dystrophi...
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PMID: 20650035
PDF is available here.
PDF is available here.
Canadian Veterinary Journal (50)12 2009
Abstract
Primary persistent atrial standstill due to atrioventricular muscle dystrophy is a rare familial disease in dogs. The diagnosis of this disorder in a 5-month-old English springer spaniel is the earliest in dogs that have been presented at the Ontario Veterinary College.
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PMID: 20190980
PDF is available here.
PDF is available here.
FEBS Letters (583)22 2009
Abstract
Duchenne myopathy is a lethal disease due to the absence of dystrophin, a cytoskeletal protein. Muscles from dystrophin-deficient mice (mdx) typically present an exaggerated susceptibility to eccentric work characterized by an important force drop and an increased membrane permeability consecutive t...
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PMID: 19840792
PDF is available here.
PDF is available here.
FASEB Journal (23)8 2009
Abstract
We treated mdx mice with intraperitoneal injections of imatinib at the peak of limb muscle inflammation and the onset of diaphragm fibrosis. Controls received PBS vehicle or were left untreated. Muscle necrosis, inflammation, fibrosis, and function were evaluated by measuring serum CK activity, endo...
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PMID: 19289603
PDF is available here.
PDF is available here.
Gene Therapy (16)6 2009
Abstract
We examined the use of a secreted form of the mouse alkaline phosphatase (mSeAP) enzyme as the reporter system transduced into MPCs using a retroviral vector. We show that circulating mSeAP could be detected in the serum of the transplanted mice at different time points after MPC transplantation. We...
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PMID: 19282846
PDF is available here.
PDF is available here.
Abstract
To investigate the differentiation of rat bone marrow mesenchymal stem cells (MSCs) into myocytes and their expression of dystrophin/utrophin after transplantation in mdx mice.
BrdU-labeled fifth-passage rat MSCs were transplanted in mdx mice with previous total body...
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PMID: 19460724
PDF is available here.
PDF is available here.
Abstract
We show that using cationic polymethylmethacrylate (PMMA) (marked as T1) nanoparticles loaded with a low dose of 2'-O-methyl-phosphorothioate (2'OMePS) AON delivered by weekly intraperitoneal (IP) injection (0.9 mg/kg/week), could restore dystrophin expression in body-wide striated muscles. Delivery...
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PMID: 19240694
PDF is available here.
PDF is available here.
Abstract
We wished to determine whether dystrophin functions in subcellular localization of nNOS and which regions may be necessary. Using in vivo transfection of dystrophin deletion constructs, we show that sarcolemmal targeting of nNOS was dependent on the spectrin-like repeats 16 and 17 (R16/17) within th...
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PMID: 19229108
PDF is available here.
PDF is available here.
Abstract
NOS is a key enzyme in the production of NO, a molecule that directly regulates vasorelaxation and blood supply. Diverse forms of muscle disease have been clinically associated with unusual fatigue after exercise. The localization of neuronal NOS (nNOS) at the plasma membrane of muscle has recently...
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PMID: 19306501
PDF is available here.
PDF is available here.
Proteomics (9)3 2009
Abstract
We could show the reversal of secondary pathobiochemical abnormalities in the dystrophic diaphragm following exon-23 skipping. In analogy to the restoration of dystrophin, beta-dystroglycan and neuronal nitric oxide synthase, the muscular dystrophy-associated differential expression of calsequestrin...
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PMID: 19132684
PDF is available here.
PDF is available here.
Abstract
We have isolated and cultured human primordial germ cells (PGCs) from early embryos. The PGCs expressed embryonic germ (EG) cell-specific surface markers, including Oct4 and Nanos. We derived a cell population from these PGCs that we termed embryoid body-derived (EBD) cells. EBD cells can be extensi...
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PMID: 19071088
PDF is available here.
PDF is available here.
Abstract
Cell Transplantation (18)7 2009
Abstract
We used a lentivirus to overexpress the follistatin protein in normal myoblasts to block the myostatin signaling. We measured the potential of transduced myoblasts to proliferate and to form multinucleated myotubes in vitro. And finally, we considered the engraftment success of those transduced myob...
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PMID: 19520047
PDF is available here.
PDF is available here.
Human Molecular Genetics (17)24 2008
Abstract
We demonstrate widespread systemic correction of dystrophin expression in body-wide muscles and cardiac tissue in adult dystrophic mdx mice, with a single low-dose injection of peptide-conjugated PMO AO. This approach was sufficient to restore uniform, high-level dystrophin protein expression in per...
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PMID: 18784278
PDF is available here.
PDF is available here.
Abstract
Ullrich Congenital Muscular Dystrophy (UCMD) and Bethlem Myopathy (BM) are muscle diseases due to mutations in the genes encoding the extracellular matrix protein collagen VI. Generation of a dystrophic mouse model where collagen VI synthesis was prevented by genetic ablation of the Col6a1 gene allo...
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PMID: 19076452
PDF is available here.
PDF is available here.
Abstract
We have determined whether the 8 month old mdx mouse, an animal model of muscular dystrophy, also has abnormal cardiac function and energetics. In vivo cardiac MRI revealed 33% and 104% larger right ventricular end-diastolic and end-systolic volumes, respectively, and 17% lower right ventricular eje...
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PMID: 18929569
PDF is available here.
PDF is available here.
Abstract
Gadofluorine M enhancement was significantly greater in skeletal muscle of 30-week-old mice with dysferlin-deficient muscular dystrophy, compared with control mice. Gadofluorine M demonstrated both increased rate of enhancement (PS rho sec(-1) +/- standard error of the mean: 0.004 e(-)(4) +/- 3 vs 0...
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PMID: 19001151
PDF is available here.
PDF is available here.
Abstract
Abstract
We hypothesized that the N2A deletion would negatively impact the force-generating capacity and passive mechanical properties of the mdm diaphragm. We measured in vitro active isometric contractile and passive length-tension properties to assess muscle function at 2 and 6 wk of age. Micro-CT, myosin...
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PMID: 18753318
PDF is available here.
PDF is available here.
Neuromuscular Disorders (18)11 2008
Abstract
Neurobiology of Disease (32)2 2008
Abstract
We observed an increase in dystrophin expression level in all the fibers, although lower than that observed in normal fibers, and found a concomitant recovery of aquaporin-4 at sarcolemma. A significant reduction in centronucleated fibers, in the area of necrosis and in the percentage of nuclear fac...
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PMID: 18694830
PDF is available here.
PDF is available here.
Neuromuscular Disorders (18)11 2008
Abstract
Neuromuscular Disorders (18)11 2008
Abstract
We evaluated the effect of halofuginone, a collagen synthesis inhibitor, on collagen synthesis in various muscles of young wild-type (C57/BL/6J) and mdx mice. Halofuginone prevented the age-dependent increase in collagen synthesis in the diaphragms of mdx with no effect on wild-type mice (n = 5 for...
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PMID: 18672370
PDF is available here.
PDF is available here.
Abstract
In vitro investigations showed protective effect of low concentrations of Debio 025 against cell death. Histology demonstrated that Debio 025 partially protected the diaphragm and soleus muscles against necrosis (10 and 100 mg kg(-1), respectively). Hindlimb muscles from mice receiving Debio 025 at...
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PMID: 18641676
PDF is available here.
PDF is available here.
Muscle & Nerve (38)4 2008
Abstract
We found that some, but not all, of the expected adaptations occurred in mdx mouse muscle. We must better understand these (non)adaptations in order to inform individuals with DMD about the benefits of exercise....
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PMID: 18816601
PDF is available here.
PDF is available here.
New England Journal of Medicine (359)13 2008
Abstract
Abstract
We isolated a population of adult muscle mononuclear cells (AMMCs) from normal, strain-matched muscle and transplanted these cells into delta-sarcoglycan-null dystrophic mice. Distinct from other transplant studies, the recipient mice were immunocompetent with an intact endogenous satellite cell poo...
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PMID: 18711004
PDF is available here.
PDF is available here.
Tijdschrift voor diergeneeskunde (133)17 2008
Abstract
Within a week of being turned out to pasture, 3 of 30 heifers displayed a symptom described in the literature as "Flying Scapulas". The cause of this symptom is severe muscular dystrophy, with subsequent rupture of the ventral serrate muscles. Blood analysis revealed a deficiency of both vitamin E a...
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PMID: 18807612
PDF is available here.
PDF is available here.
Human Molecular Genetics (17)17 2008
Abstract
We hypothesized that the pathology observed with dystrophin deficiency may be developmentally regulated. Initially, we demonstrated that profound small interfering RNA-mediated dystrophin knockdown could be achieved in mouse primary muscle cultures. The use of adeno-associated virus vectors to expre...
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PMID: 18511456
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (283)36 2008
Abstract
We performed gene expression profiling of laminin alpha2 chain-deficient mouse limb muscle. One of the down-regulated genes encodes a protein called Cib2 (calcium- and integrin-binding protein 2) whose expression and function is unknown. However, the closely related Cib1 has been reported to bind in...
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PMID: 18611855
PDF is available here.
PDF is available here.
Human Molecular Genetics (17)16 2008
Abstract
We explored a method whereby new myoblasts could be generated in dystrophic muscles by transplantation of primary fibroblasts engineered to express a micro-dystrophin/enhanced green fluorescent protein (muDys/eGFP) fusion gene together with a tamoxifen-inducible form of the myogenic regulator MyoD [...
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PMID: 18511457
PDF is available here.
PDF is available here.
Human Gene Therapy (19)8 2008
Abstract
We show that a single intravenous injection of AAV serotype-9 (AAV-9) microdystrophin vector efficiently transduced the entire heart in neonatal mdx mice, a dystrophin-deficient mouse DMD model. Furthermore, microdystrophin therapy normalized the heart rate, PR interval, and QT interval. The cardiom...
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PMID: 18666839
PDF is available here.
PDF is available here.
Human Gene Therapy (19)8 2008
Abstract
We previously established that copolymers made of poly(ethylene imine) (PEI) and poly(ethylene glycol) (PEG) enhanced ESO transfection in skeletal muscle of mdx mice, resulting in widespread distribution of dystrophin-positive fibers, but limited dystrophin expression by Western blot. In an attempt...
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PMID: 18647087
PDF is available here.
PDF is available here.
Gene Therapy (15)15 2008
Abstract
We report the effectiveness of in vivo siRNA delivery into skeletal muscles of normal or diseased mice through nanoparticle formation of chemically unmodified siRNAs with atelocollagen (ATCOL). ATCOL-mediated local application of siRNA targeting myostatin, a negative regulator of skeletal muscle gro...
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PMID: 18323791
PDF is available here.
PDF is available here.
Gene Therapy (15)15 2008
Abstract
We wish to use the dystrophin-deficient golden retriever muscular dystrophy (GRMD) dog, a canine model of DMD, to investigate adeno-associated virus (AAV) vector-mediated minidystrophin gene therapy. The dog model is useful in evaluating vector dose requirement and immunological consequences owing t...
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PMID: 18432277
PDF is available here.
PDF is available here.
Abstract
We developed an efficient selection for heritable mitochondrial genome (mtDNA) mutations in Drosophila, thereby enhancing a metazoan model for study of mitochondrial genetics and mutations causing human mitochondrial disease. Targeting a restriction enzyme to mitochondria in the germline compromised...
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PMID: 18653897
PDF is available here.
PDF is available here.
Abstract
We used cell-surface marker expression to purify from the satellite cell pool a distinct population of skeletal muscle precursors (SMPs) that function as muscle stem cells. When engrafted into muscle of dystrophin-deficient mdx mice, purified SMPs contributed to up to 94% of myofibers, restoring dys...
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PMID: 18614009
PDF is available here.
PDF is available here.
Abstract
Progressive muscle weakness beginning at 6 months of age was observed in a male Persian-mix cat. Muscle atrophy and joint contracture progressed over the next 3 years. The cat had developed gait difficulty at 8 months of age. The cat died at age of 5 years and 3 months due to an acute respiratory di...
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PMID: 18243745
PDF is available here.
PDF is available here.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology / edited by the Gaetano Conte Academy for the study of striated muscle diseases (27) 2008
Abstract
We have developed a myostatin-specific inhibitor derived from follistatin, designated FS I-I. Transgenic mice expressing this myostatin-inhibiting peptide under the control of a skeletal muscle-specific promoter showed increased skeletal muscle mass and strength. mdx mice were crossed with FS I-I tr...
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PMID: 19108572
PDF is available here.
PDF is available here.
Molecules and Cells (25)4 2008
Abstract
We evaluated the efficacy of curcumin, a potent NF-kappaB inhibitor, in mdx mice, a mouse model of DMD. We found that it improved sarcolemmic integrity and enhanced muscle strength after intraperitoneal (i.p.) injection. Histological analysis revealed that the structural defects of myofibrils were r...
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PMID: 18460899
PDF is available here.
PDF is available here.
Brain Pathology (18)3 2008
Abstract
We examined the myonuclei of LMNA null mice at the myotendinous junctions (MTJ), the site of longitudinal force transmission from contractile proteins to extracellular proteins. The right soleus and rectus femoris muscles of five null mutants aged 5-7 weeks and two wild-type animals aged 5 weeks and...
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PMID: 18371185
PDF is available here.
PDF is available here.
Genes & Development (22)13 2008
Abstract
We show that fibrinogen accumulates in dystrophic muscles of DMD patients and mdx mice. Genetic loss or pharmacological depletion of fibrinogen in these mice reduced fibrosis and dystrophy progression. Our results demonstrate that fibrinogen-Mac-1 receptor binding, through induction of IL-1beta, dri...
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PMID: 18593877
PDF is available here.
PDF is available here.
Transplantation (85)12 2008
Abstract
Central tolerance achieved using regimens that eliminate nearly all endogenous peripheral lymphocytes (i.e., lethal irradiation) or a nonmyeloablative protocol that depleted peripheral CD8 cells, results in lymphocytic infiltration in muscles that received MT but not in skin allografts. This suggest...
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PMID: 18580473
PDF is available here.
PDF is available here.
FEBS Letters (582)15 2008
Abstract
We performed sequence comparison and gene expression analysis to elucidate the responsible gene. One missense mutation was detected in AM candidate genes, while no remarkable alteration of expression patterns was observed. The mutation was identified in WWP1, detected only in dystrophic chickens wit...
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PMID: 18501710
PDF is available here.
PDF is available here.
Abstract
We have focused our efforts on the derivation of skeletal muscle progenitors from ES cells and here we will discuss the strategy of reprogramming lineage choices by overexpression of a master regulator, which has proven successful for the generation of the skeletal myogenic lineage from mouse ES cel...
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PMID: 18583932
PDF is available here.
PDF is available here.
Neuromuscular Disorders (18)6 2008
Abstract
We studied gene expression and localization of non-voltage gated cation channels in normal and mdx mouse skeletal muscle. We found TRPC3, TRPC6, TRPV4, TRPM4 and TRPM7 to be the most abundant isoforms. Immunofluorescent staining of muscle cross-sections with antibodies against TRP proteins showed sa...
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PMID: 18504127
PDF is available here.
PDF is available here.
Circulation (117)19 2008
Abstract
Selective vacuolar degeneration of Purkinje fibers was found in the early stages of dystrophin deficiency. Dislocation of utrophin besides upregulation of Dp71 can be involved with this pathology. The degeneration of Purkinje fibers can be associated with the distinct deep Q waves in ECG and fatal a...
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PMID: 18458171
PDF is available here.
PDF is available here.
Abstract
We compared muscle force and pathology in mdx3cv and mdx4cv mice. Dystrophin was eliminated in mdx4cv mouse muscle but was expressed in mdx3cv mice as a near full-length protein at approximately 5% of normal levels. Consistent with previous reports, we found dystrophic muscle pathology in both mouse...
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PMID: 18385524
PDF is available here.
PDF is available here.
Human Molecular Genetics (17)9 2008
Abstract
We have introduced in mice the most prevalent LGMD2D mutation, R77C. It should be noted that the natural murine residue at this position is a histidine. The model is, therefore, referred as Sgca(H77C/H77C). Unexpectedly, we observed an absence of LGMD2D-like phenotype at histological or physiologica...
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PMID: 18252745
PDF is available here.
PDF is available here.