A fusion peptide directs enhanced systemic dystrophin exon skipping and functional restoration in dystrophin-deficient mdx mice...
AO directs highly efficient systemic dystrophin splice correction in mdx mice. With very low systemic doses, we demonstrate ... have been shown to skip the mutated exon and partially restore functional dystrophin protein in dystrophin-deficient mdx mice. ... yielding functional correction and improvement of the mdx dystrophic phenotype. Our data demonstrate proof-of-concept for this ... Mice, Inbred mdx, Molecular Sequence Data, Morpholines, Morpholinos, Muscular Dystrophy, Duchenne, Oligonucleotides, Antisense ...https://www.neuroscience.ox.ac.uk/publications/113439
SERCA2a gene transfer improves electrocardiographic performance in aged mdx mice.
Mice. Mice, Inbred mdx. Myocardium / metabolism, pathology. Sarcoplasmic Reticulum Calcium-Transporting ATPases / genetics*, ... The vector genome was detected in all mdx mice that received AAV-9 SERCA2a injection but not in untreated mdx mice (Figure 1B ... Compared to that of age- and gender-matched untreated mdx mice, the ECG profile of AAV-9 SERCA2a treated mdx mice was ... Since the heart of young mdx mice is mildly affected, we opted to test SERCA2a therapy in 12-month-old mdx mice . At this ...http://www.biomedsearch.com/nih/SERCA2a-gene-transfer-improves-electrocardiographic/21834967.html
CoQ10 and Prednisone in Non-Ambulatory DMD
Mice, Inbred Mdx. A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. This mutation is X ... The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR ...https://www.bioportfolio.com/resources/trial/108534/CoQ10-and-Prednisone-in-Non-Ambulatory-DMD.html
Pip5 transduction peptides direct high efficiency oligonucleotide-mediated dystrophin exon skipping in heart and phenotypic...
Animals, Blotting, Western, Exons, Immunohistochemistry, Mice, Mice, Inbred mdx, Muscular Dystrophy, Duchenne, Myocardium, ... One of the lead peptide-AO conjugates, Pip5e-AO, showed highly efficient exon skipping and dystrophin production in mdx mice ... Antisense oligonucleotide (AO)-mediated exon skipping has been shown to restore functional dystrophin in mdx mice and DMD ... direct high efficiency oligonucleotide-mediated dystrophin exon skipping in heart and phenotypic correction in mdx mice. Mol ...http://epubs.surrey.ac.uk/825962/
Detection of dystrophin with exon specific antibodies. | Open-i
Mice, Inbred mdx. *Muscular Dystrophy, Duchenne/genetics. *MutS Homolog 2 Protein/metabolism ... The mdx5cv mouse is a model of DMD in which a point mutation in exon 10 of the dystrophin gene creates an artificial splice ... The mdx5cv mouse is a model of DMD in which a point mutation in exon 10 of the dystrophin gene creates an artificial splice ... Bottom Line: The mdx5cv mouse is a model of DMD in which a point mutation in exon 10 of the dystrophin gene creates an ...https://openi.nlm.nih.gov/detailedresult.php?img=PMC2654480_1471-2199-10-15-4&req=4
Hannah Crabb - Research Output - the UWA Profiles and Research Repository
Reduced muscle necrosis and long-term benefits in dystrophic mdx mice after cVIq (blockade of TNF) treatment. Radley, H. G., ... A single 30 min treadmill exercise session is suitable for 'proof-of concept studies' in adult mdx mice: A comparison of the ... Cromolyn administration (to block mast cell degranulation) reduces necrosis of dystrophic muscle in mdx mice. Radley, H. & ... Reduced necrosis of dystrophic muscle by depletion of host neutrophils, or blocking TNFα function with Etanercept in mdx mice. ...https://research-repository.uwa.edu.au/en/persons/hannah-crabb/publications/
Inbred C57BL Mouse Medicine & Life Sciences Facioscapulohumeral Muscular Dystrophy Medicine & Life Sciences ... Expression patterns of regulatory RNAs, including lncRNAs and tRNAs, during postnatal growth of normal and dystrophic (mdx) ... mouse muscles, and their response to taurine treatment. Butchart, L. C., Terrill, J. R., Rossetti, G., White, R., Filipovska, A ... sarcopenia and increases markers of mitochondrial function and autophagy in muscles of old male and female C57BL/6J mice. [ ...https://research-repository.uwa.edu.au/en/persons/robert-white
Xiao Xiao - Publications - University of North Carolina at Chapel Hill
Improvement of the mdx mouse dystrophic phenotype by systemic in utero AAV8 delivery of a minidystrophin gene. Koppanati, B. M ... One-year treatment of morpholino antisense oligomer improves skeletal and cardiac muscle functions in dystrophic mdx mice. Wu, ... Mouse models of fukutin-related protein mutations show a wide range of disease phenotypes. Blaeser, A., Keramaris, E., Chan, Y ... Overcoming insulin insufficiency by forced follistatin expression in β-cells of db/db mice. Zhao, C., Qiao, C., Tang, R. H., ...https://uncch.pure.elsevier.com/en/persons/xiao-xiao/publications/?type=%2Fdk%2Fatira%2Fpure%2Fresearchoutput%2Fresearchoutputtypes%2Fcontributiontojournal%2Farticle
List of MeSH codes (B01) - Wikipedia
... mice, inbred c3h MeSH B01.050.157.520.420 --- mice, inbred c57bl MeSH B01.050.157.520.420.500 --- mice, inbred mdx MeSH B01.050 ... mice, inbred c3h MeSH B01.050.199.520.520.420 --- mice, inbred c57bl MeSH B01.050.199.520.520.420.500 --- mice, inbred mdx MeSH ... mice, inbred a MeSH B01.050.157.520.318 --- mice, inbred akr MeSH B01.050.157.520.338 --- mice, inbred balb c MeSH B01.050. ... mice, inbred hrs MeSH B01.050.157.520.510 --- mice, inbred icr MeSH B01.050.157.520.555 --- mice, inbred mrl lpr MeSH B01.050. ...https://en.wikipedia.org/wiki/List_of_MeSH_codes_(B01)
NAVER Academic > Search...
Animals, Calcium, metabolism, Homeostasis, physiology, Membranes, ultrastructure, Mice, Mice, Inbred mdx, Muscles, Muscular ... Membrane abnormalities and Ca homeostasis in muscles of the mdx mouse, an animal model of the Duchenne muscular dystrophy: a ...https://academic.naver.com/search.naver?field=3&query=Acta+physiologica+Scandinavica+156%EA%B6%8C+3%ED%98%B8
An in vivo and in vitro H-magnetic resonance spectroscopy study of mdx mouse brain: abnormal development or neural necrosis? -...
Control mice were compared to mdx using a combination of in vivo and in vitro 1H-MRS methods to determine whether neural ... of both DMD children and the mdx mouse model. The underlying biochemical lesion causing mental impairment in DMD is unknown. 1H ... NAA levels were normal in mdx brain compared to controls suggesting minor, if any, neuronal necrosis in dystrophic brain. In ... Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Necrosis, Neurons ... Control mice were compared to mdx using a combination of in ...https://www.win.ox.ac.uk/publications/376237
Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy....
Mice, Inbred C57BL, Mice, Inbred mdx, Morpholinos, Muscle, Skeletal, Muscular Dystrophy, Duchenne, NADPH Oxidase 4, NADPH ... Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy. ... Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy. ...https://www.dpag.ox.ac.uk/publications/528590
Bi-specific splice-switching PMO oligonucleotides conjugated via a single peptide active in a mouse model of Duchenne muscular...
... for both pre-mRNA targets when compared to individual CPP-PMO conjugates both in cell culture and in vivo in the mdx mouse ... in a mouse model of Duchenne muscular dystrophy. Conjugations of PMOs to a single CPP were carried out through an amide bond in ... Mice, Mice, Inbred mdx, Morpholinos, Muscular Dystrophy, Duchenne, RNA Splicing ... for both pre-mRNA targets when compared to individual CPP-PMO conjugates both in cell culture and in vivo in the mdx mouse ...https://www.neuroscience.ox.ac.uk/publications/493318
Extracellular microRNAs are dynamic non-vesicular biomarkers of muscle turnover. - Nuffield Department of Obstetrics and...
... show dynamic patterns of expression that mirror the progression of muscle pathology in mdx mice. Expression of the myogenic ... In this study, serum miRNA profiling reveals a distinct extracellular miRNA signature in dystrophin-deficient mdx mice, which ... dystromiRs were elevated following experimentally-induced skeletal muscle injury and regeneration in non-dystrophic mice. Only ... Animals, Biomarkers, Blood Proteins, Male, Mice, Mice, Inbred C57BL, Mice, Inbred mdx, MicroRNAs, Muscle, Skeletal, Muscular ...https://www.obs-gyn.ox.ac.uk/publications/418135
Assembly of multiple dystrobrevin-containing complexes in the kidney. - Nuffield Department of Clinical Neurosciences
... findings also provide a possible explanation for the lack of kidney pathology in Duchenne muscular dystrophy patients and mice ... the molecular architecture of dystrophin-like protein complexes in kidneys from normal and dystrophin-deficient mice. Using ... Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Muscle Proteins, Nephrons, Utrophin ... findings also provide a possible explanation for the lack of kidney pathology in Duchenne muscular dystrophy patients and mice ...https://www.ndcn.ox.ac.uk/publications/106239
Prevention of muscular dystrophy in mice by CRISPR/Cas9-mediated editing of germline DNA. - PubMed - NCBI
Mice, Inbred mdx. *Muscle, Skeletal/pathology. *Muscular Dystrophy, Duchenne/genetics. *Muscular Dystrophy, Duchenne/pathology ... A) Schematic of the targeted exon of mouse Dmd and sequence from wild-type (upper) and mdx mice (lower).The mdx point mutation ... Strategy of the gene correction in mdx mice via germline gene therapy. (E) Genotyping results of mdx-C mice with mosaicism of 2 ... Immunostaining and histological analysis of muscles from 7- to 9-week-old wild-type, mdx, and mdx-C mice (HDR-17%, HDR-41%, or ...https://www.ncbi.nlm.nih.gov/pubmed/25123483
Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies. - Semantic Scholar
... a dystrophinopathy mouse model commonly used in pre-clinical studies. mdx mice ambulated shorter distances than normal controls ... To address this, a six-minute ambulation assay, serum creatine kinase, and myoglobinuria were investigated for the mdx mouse, ... An asymmetric pathophysiology in mdx mice was unmasked with exercise, and peak measurements of serum creatine kinase and ... Testing of SHIRPA, a mouse phenotypic assessment protocol, on Dmd mdx and Dmd mdx3cv dystrophin-deficient mice. *J. A. S. ...https://www.semanticscholar.org/paper/Endpoint-measures-in-the-mdx-mouse-relevant-for-Kobayashi-Rader/d3bd7246d5b411eb8003991faa9ea75fc0a6cedf
Christopher F Spurney
inbred mdx mice*nadph oxidase*dilated cardiomyopathy*duchenne muscular dystrophy*dystrophin*extracellular matrix proteins* ... Glucocorticoid-treated mice are an inappropriate positive control for long-term preclinical studies in the mdx mouse. Arpana ... inbred c57bl mice*animal disease models*reverse transcriptase polymerase chain reaction*ventricular pressure*aortic valve* ... Functional and molecular effects of arginine butyrate and prednisone on muscle and heart in the mdx mouse model of Duchenne ...https://www.labome.org/expert/usa/childrens/spurney/christopher-f-spurney-1003395.html
Identification of novel, therapy-responsive protein biomarkers in a mouse model of Duchenne muscular dystrophy by aptamer-based...
... mice. The serum levels of 96 proteins were found to be significantly altered (P , 0.001, q , 0.01) in mdx mice. Additionally, ... This study has identified multiple novel, therapy-responsive protein biomarkers in the serum of the mdx mouse with potential ... Here we have utilised an aptamer-based proteomics approach to profile 1,129 proteins in the serum of wild-type and mdx ( ... were validated by ELISA in the mouse samples. Furthermore, ADAMTS5 was found to be significantly elevated in human DMD patient ...https://www.dpag.ox.ac.uk/publications/579560
Activation of protein phosphatase-1 isoforms and glycogen synthase kinase-3 beta in muscle from mdx mice. - Semantic Scholar
We investigated the activities and protein levels of the three PP1 isoforms and of GSK-3 in muscle of mdx dystrophic mice. PP1 ... On the other hand, the immunodetected PP1 delta was lower in the glycogen and microsomal fractions from mdx muscle. GSK-3 was ... The changes described herein may be related to the pathological events occurring in the mdx muscle. These include increased ... Higher PP1 activity was assayed in the cytosol from mdx rather than from control muscles. Immunoprecipitation indicated that ...https://www.semanticscholar.org/paper/Activation-of-protein-phosphatase-1-isoforms-and-in-Villa-Moruzzi-Puntoni/7adf56f32a3e511b3fbddfe00f2a00c5193476f4
Exclusive skeletal muscle correction does not modulate dystrophic heart disease in the aged mdx model of Duchenne...
... mdx and transgenic mdx mice. N = 17 for BL10, N = 9 for mdx and N = 6 for transgenic mdx mice. The P-values of the Bonferroni ... Mice, Inbred mdx. *Muscle, Skeletal/metabolism. *Muscle, Skeletal/pathology. *Muscular Dystrophy, Duchenne/complications* ... Studies performed in young adult mdx mice (a mild DMD mouse model) have yielded opposing results. Since mdx mice do not develop ... As expected, transgenic mdx mice had minimal skeletal muscle disease and they also outperformed original mdx mice on treadmill ...https://www.ncbi.nlm.nih.gov/pubmed/23459935
Macroscopic and microscopic evaluation of EBD staining | Open-i
... mdx (c and d), and dy/dy (e and ... Mice. *Mice, Inbred mdx. *Mice, Mutant Strains. *Mice, ... In contrast to mdx mice (c), dy/dy (e) or dy2J/dy2J mice never showed localized EBD uptake into skeletal muscles by visual ... In contrast to mdx mice (c), dy/dy (e) or dy2J/dy2J mice never showed localized EBD uptake into skeletal muscles by visual ... Evans blue, a low molecular weight diazo dye, does not cross into skeletal muscle fibers in normal mice. In contrast, mdx mice ...https://openi.nlm.nih.gov/detailedresult.php?img=PMC2139791_JCB.16464f6&req=4
Sulforaphane alleviates muscular dystrophy in mdx mice by activation of Nrf2. - PubMed - NCBI
Mice, Inbred C57BL. *Muscle, Skeletal/drug effects. *Muscular Dystrophy, Animal/drug therapy* ... Sulforaphane alleviates muscular dystrophy in mdx mice by activation of Nrf2.. Sun C1, Yang C2, Xue R1, Li S1, Zhang T1, Pan L1 ... Four-week-old mdx mice were treated with SFN by gavage (2 mg·kg body wt(-1)·day(-1) for 8 wk), and our results demonstrated ... of mdx mice and decreased the activities of plasma creatine phosphokinase (∼45%) and lactate dehydrogenase (∼40%), ...https://www.ncbi.nlm.nih.gov/pubmed/25593219
A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. - Physiology, Anatomy...
Animals, Blood Vessels, Cytoskeletal Proteins, Epitopes, Humans, Immunohistochemistry, Membrane Proteins, Mice, Mice, Inbred ... A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. ... A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. ... C57BL, Mice, Knockout, Muscle, Skeletal, Muscular Dystrophy, Duchenne, Myocardium, Protein Isoforms, RNA, Messenger, Tissue ...https://www.dpag.ox.ac.uk/publications/106378