A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. This mutation is X chromosome-linked and produces viable homozygous animals that lack the muscle protein DYSTROPHIN, have high serum levels of muscle ENZYMES, and possess histological lesions similar to human MUSCULAR DYSTROPHY. The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR DYSTROPHY.
An X-linked recessive muscle disease caused by an inability to synthesize DYSTROPHIN, which is involved with maintaining the integrity of the sarcolemma. Muscle fibers undergo a process that features degeneration and regeneration. Clinical manifestations include proximal weakness in the first few years of life, pseudohypertrophy, cardiomyopathy (see MYOCARDIAL DISEASES), and an increased incidence of impaired mentation. Becker muscular dystrophy is a closely related condition featuring a later onset of disease (usually adolescence) and a slowly progressive course. (Adams et al., Principles of Neurology, 6th ed, p1415)
A muscle protein localized in surface membranes which is the product of the Duchenne/Becker muscular dystrophy gene. Individuals with Duchenne muscular dystrophy usually lack dystrophin completely while those with Becker muscular dystrophy have dystrophin of an altered size. It shares features with other cytoskeletal proteins such as SPECTRIN and alpha-actinin but the precise function of dystrophin is not clear. One possible role might be to preserve the integrity and alignment of the plasma membrane to the myofibrils during muscle contraction and relaxation. MW 400 kDa.
An autosomally-encoded 376-kDa cytoskeletal protein that is similar in structure and function to DYSTROPHIN. It is a ubiquitously-expressed protein that plays a role in anchoring the CYTOSKELETON to the PLASMA MEMBRANE.
A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.
A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.
The musculofibrous partition that separates the THORACIC CAVITY from the ABDOMINAL CAVITY. Contraction of the diaphragm increases the volume of the thoracic cavity aiding INHALATION.
Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of SKELETAL MUSCLE. They consist of MYOFIBRILS enclosed within and attached to the SARCOLEMMA. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.
A group of proteins that associate with DYSTROPHIN at the CELL MEMBRANE to form the DYSTROPHIN-ASSOCIATED PROTEIN COMPLEX.
The excitable plasma membrane of a muscle cell. (Glick, Glossary of Biochemistry and Molecular Biology, 1990)
Dystrophin-associated proteins that play role in the formation of a transmembrane link between laminin-2 and DYSTROPHIN. Both the alpha and the beta subtypes of dystroglycan originate via POST-TRANSLATIONAL PROTEIN PROCESSING of a single precursor protein.
The physiological renewal, repair, or replacement of tissue.
An amino acid-specifying codon that has been converted to a stop codon (CODON, TERMINATOR) by mutation. Its occurance is abnormal causing premature termination of protein translation and results in production of truncated and non-functional proteins. A nonsense mutation is one that converts an amino acid-specific codon to a stop codon.
Any codon that signals the termination of genetic translation (TRANSLATION, GENETIC). PEPTIDE TERMINATION FACTORS bind to the stop codon and trigger the hydrolysis of the aminoacyl bond connecting the completed polypeptide to the tRNA. Terminator codons do not specify amino acids.
An mRNA metabolic process that distinguishes a normal STOP CODON from a premature stop codon (NONSENSE CODON) and facilitates rapid degradation of aberrant mRNAs containing premature stop codons.
A set of three nucleotides in a protein coding sequence that specifies individual amino acids or a termination signal (CODON, TERMINATOR). Most codons are universal, but some organisms do not produce the transfer RNAs (RNA, TRANSFER) complementary to all codons. These codons are referred to as unassigned codons (CODONS, NONSENSE).
Calcium-transporting ATPases that catalyze the active transport of CALCIUM into the SARCOPLASMIC RETICULUM vesicles from the CYTOPLASM. They are primarily found in MUSCLE CELLS and play a role in the relaxation of MUSCLES.
A network of tubules and sacs in the cytoplasm of SKELETAL MUSCLE FIBERS that assist with muscle contraction and relaxation by releasing and storing calcium ions.
Cation-transporting proteins that utilize the energy of ATP hydrolysis for the transport of CALCIUM. They differ from CALCIUM CHANNELS which allow calcium to pass through a membrane without the use of energy.
The use of statistical methods in the analysis of a body of literature to reveal the historical development of subject fields and patterns of authorship, publication, and use. Formerly called statistical bibliography. (from The ALA Glossary of Library and Information Science, 1983)
Copies of a work or document distributed to the public by sale, rental, lease, or lending. (From ALA Glossary of Library and Information Science, 1983, p181)
A publication issued at stated, more or less regular, intervals.
"The business or profession of the commercial production and issuance of literature" (Webster's 3d). It includes the publisher, publication processes, editing and editors. Production may be by conventional printing methods or by electronic publishing.
Seizures that occur during a febrile episode. It is a common condition, affecting 2-5% of children aged 3 months to five years. An autosomal dominant pattern of inheritance has been identified in some families. The majority are simple febrile seizures (generally defined as generalized onset, single seizures with a duration of less than 30 minutes). Complex febrile seizures are characterized by focal onset, duration greater than 30 minutes, and/or more than one seizure in a 24 hour period. The likelihood of developing epilepsy (i.e., a nonfebrile seizure disorder) following simple febrile seizures is low. Complex febrile seizures are associated with a moderately increased incidence of epilepsy. (From Menkes, Textbook of Child Neurology, 5th ed, p784)
A medical specialty concerned with the hypersensitivity of the individual to foreign substances and protection from the resultant infection or disorder.
Critical and exhaustive investigation or experimentation, having for its aim the discovery of new facts and their correct interpretation, the revision of accepted conclusions, theories, or laws in the light of newly discovered facts, or the practical application of such new or revised conclusions, theories, or laws. (Webster, 3d ed)
The study of the patterns of ridges of the skin of the fingers, palms, toes, and soles.
A technique for identifying individuals of a species that is based on the uniqueness of their DNA sequence. Uniqueness is determined by identifying which combination of allelic variations occur in the individual at a statistically relevant number of different loci. In forensic studies, RESTRICTION FRAGMENT LENGTH POLYMORPHISM of multiple, highly polymorphic VNTR LOCI or MICROSATELLITE REPEAT loci are analyzed. The number of loci used for the profile depends on the ALLELE FREQUENCY in the population.
Group composed of associates of same species, approximately the same age, and usually of similar rank or social status.
A form of interactive entertainment in which the player controls electronically generated images that appear on a video display screen. This includes video games played in the home on special machines or home computers, and those played in arcades.
Recording of visual and sometimes sound signals on magnetic tape.
A quantitative measure of the frequency on average with which articles in a journal have been cited in a given period of time.
The evaluation by experts of the quality and pertinence of research or research proposals of other experts in the same field. Peer review is used by editors in deciding which submissions warrant publication, by granting agencies to determine which proposals should be funded, and by academic institutions in tenure decisions.
An organized procedure carried out by a select committee of professionals in evaluating the performance of other professionals in meeting the standards of their specialty. Review by peers is used by editors in the evaluation of articles and other papers submitted for publication. Peer review is used also in the evaluation of grant applications. It is applied also in evaluating the quality of health care provided to patients.
A macromolecular complex of proteins that includes DYSTROPHIN and DYSTROPHIN-ASSOCIATED PROTEINS. It plays a structural role in the linking the CYTOSKELETON to the EXTRACELLULAR MATRIX.
Research that involves the application of the natural sciences, especially biology and physiology, to medicine.
A microcomputer-based software package providing a user-friendly interface to the MEDLARS system of the National Library of Medicine.
The scientific disciplines concerned with the embryology, anatomy, physiology, biochemistry, pharmacology, etc., of the nervous system.

Hindlimb immobilization applied to 21-day-old mdx mice prevents the occurrence of muscle degeneration. (1/735)

Dystrophin-deficient skeletal muscles of mdx mice undergo their first rounds of degeneration-regeneration at the age of 14-28 days. This feature is thought to result from an increase in motor activity at weaning. In this study, we hypothesize that if the muscle is prevented from contracting, it will avoid the degenerative changes that normally occur. For this purpose, we developed a procedure of mechanical hindlimb immobilization in 3-wk-old mice to restrain soleus (Sol) and extensor digitorum longus (EDL) muscles in the stretched or shortened position. After a 14-day period of immobilization, the striking feature was the low percentage of regenerated (centronucleated) myofibers in Sol and EDL muscles, regardless of the length at which they were fixed, compared with those on the contralateral side (stretched Sol: 8.4 +/- 6.5 vs. 46.6 +/- 10.3%, P = 0.0008; shortened Sol: 1.2 +/- 1.6 vs. 50.4 +/- 16.4%, P = 0.0008; stretched EDL: 05 +/- 0.5 vs. 32.9 +/- 17.5%, P = 0. 002; shortened EDL: 3.3 +/- 3.1 vs. 34.7 +/- 11.1%, P = 0.002). Total numbers of myofibers did not change with immobilization. This study shows that limb immobilization prevents the occurrence of the first round of myofiber necrosis in mdx mice and suggests that muscle contractions play a role in the skeletal muscle degeneration of dystrophin-deficient mdx mouse muscles.  (+info)

Increased calcium entry into dystrophin-deficient muscle fibres of MDX and ADR-MDX mice is reduced by ion channel blockers. (2/735)

1. Single fibres were enzymatically isolated from interosseus muscles of dystrophic MDX mice, myotonic-dystrophic double mutant ADR-MDX mice and C57BL/10 controls. The fibres were kept in cell culture for up to 2 weeks for the study of Ca2+ homeostasis and sarcolemmal Ca2+ permeability. 2. Resting levels of intracellular free Ca2+, determined with the fluorescent Ca2+ indicator fura-2, were slightly higher in MDX (63 +/- 20 nM; means +/- s.d.; n = 454 analysed fibres) and ADR-MDX (65 +/- 12 nM; n = 87) fibres than in controls (51 +/- 20 nM; n = 265). 3. The amplitudes of electrically induced Ca2+ transients did not differ between MDX fibres and controls. Decay time constants of Ca2+ transients ranged between 10 and 55 ms in both genotypes. In 50 % of MDX fibres (n = 68), but in only 20 % of controls (n = 54), the decay time constants were > 35 ms. 4. Bath application of Mn2+ resulted in a progressive quench of fura-2 fluorescence emitted from the fibres. The quench rate was about 2 times higher in MDX fibres (3.98 +/- 1.9 % min-1; n = 275) than in controls (2.03 +/- 1.4 % min-1; n = 204). The quench rate in ADR-MDX fibres (2.49 +/- 1.4 % min-1; n = 87) was closer to that of controls. 5. The Mn2+ influx into MDX fibres was reduced to 10 % by Gd3+, to 19 % by La3+ and to 47 % by Ni2+ (all at 50 microM). Bath application of 50 microM amiloride inhibited the Mn2+ influx to 37 %. 6. We conclude that in isolated, resting MDX muscle fibres the membrane permeability for divalent cations is increased. The presumed additional influx of Ca2+ occurs through ion channels, but is well compensated for by effective cellular Ca2+ transport systems. The milder dystrophic phenotype of ADR-MDX mice is correlated with a smaller increase of their sarcolemmal Ca2+ permeability.  (+info)

Dynamics of myoblast transplantation reveal a discrete minority of precursors with stem cell-like properties as the myogenic source. (3/735)

Myoblasts, the precursors of skeletal muscle fibers, can be induced to withdraw from the cell cycle and differentiate in vitro. Recent studies have also identified undifferentiated subpopulations that can self-renew and generate myogenic cells (Baroffio, A., M. Hamann, L. Bernheim, M.-L. Bochaton-Pillat, G. Gabbiani, and C.R. Bader. 1996. Differentiation. 60:47-57; Yoshida, N., S. Yoshida, K. Koishi, K. Masuda, and Y. Nabeshima. 1998. J. Cell Sci. 111:769-779). Cultured myoblasts can also differentiate and contribute to repair and new muscle formation in vivo, a capacity exploited in attempts to develop myoblast transplantation (MT) for genetic modification of adult muscle. Our studies of the dynamics of MT demonstrate that cultures of myoblasts contain distinct subpopulations defined by their behavior in vitro and divergent responses to grafting. By comparing a genomic and a semiconserved marker, we have followed the fate of myoblasts transplanted into muscles of dystrophic mice, finding that the majority of the grafted cells quickly die and only a minority are responsible for new muscle formation. This minority is behaviorally distinct, slowly dividing in tissue culture, but rapidly proliferative after grafting, suggesting a subpopulation with stem cell-like characteristics.  (+info)

Extensive but coordinated reorganization of the membrane skeleton in myofibers of dystrophic (mdx) mice. (4/735)

We used immunofluorescence techniques and confocal imaging to study the organization of the membrane skeleton of skeletal muscle fibers of mdx mice, which lack dystrophin. beta-Spectrin is normally found at the sarcolemma in costameres, a rectilinear array of longitudinal strands and elements overlying Z and M lines. However, in the skeletal muscle of mdx mice, beta-spectrin tends to be absent from the sarcolemma over M lines and the longitudinal strands may be disrupted or missing. Other proteins of the membrane and associated cytoskeleton, including syntrophin, beta-dystroglycan, vinculin, and Na,K-ATPase are also concentrated in costameres, in control myofibers, and mdx muscle. They also distribute into the same altered sarcolemmal arrays that contain beta-spectrin. Utrophin, which is expressed in mdx muscle, also codistributes with beta-spectrin at the mutant sarcolemma. By contrast, the distribution of structural and intracellular membrane proteins, including alpha-actinin, the Ca-ATPase and dihydropyridine receptors, is not affected, even at sites close to the sarcolemma. Our results suggest that in myofibers of the mdx mouse, the membrane- associated cytoskeleton, but not the nearby myoplasm, undergoes widespread coordinated changes in organization. These changes may contribute to the fragility of the sarcolemma of dystrophic muscle.  (+info)

The timing between skeletal muscle myoblast replication and fusion into myotubes, and the stability of regenerated dystrophic myofibres: an autoradiographic study in mdx mice. (5/735)

In mdx mice, a model for Duchenne muscular dystrophy, the timing between the replication of myoblasts and their incorporation into myotubes was determined autoradiographically. Thirty-eight mdx mice aged 23 d were injected with tritiated thymidine to label myoblasts replicating early in the dystrophic process. At intervals from 8 h to 30 d after injection the tibialis anterior muscles were removed, processed for autoradiography and analysed for labelled central myonuclei (derived from the progeny of myoblasts which had been labelled at 23 d). At 8 h after injection there were no labelled central myonuclei, showing that the labelled myoblasts had not fused within this time. At 1 d, 2 % of central myonuclei were labelled, at 2 d, up to 32% were labelled, at 3 d approximately 60% were labelled, and at 4 d the labelling peaked at 74%. In the 27 mice sampled from 5-30 d after injection, the levels of central myonuclear labelling varied enormously: from 1-63%. However, there was a consistent decrease in the numbers of labelled central myonuclei with time. This may have been due to dilution of the relative numbers of labelled myonuclei due to other, nonlabelled, myoblasts replicating after the availability of tritiated thymidine, and fusing. It was also possible that labelled myofibres underwent subsequent necrosis and were eliminated from the muscle. The proposal that a regenerated myofibre can undergo a subsequent cycle of necrosis and regeneration was supported by evidence of some necrotic myofibres with labelled and unlabelled central nuclei. These results have implications for understanding the cellular biology and pathology of dystrophic muscle, particularly in relation to myoblast transfer therapy as a potential treatment of Duchenne muscular dystrophy.  (+info)

Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice. (6/735)

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene, leading to the absence of the dystrophin protein in striated muscle. A significant number of these mutations are premature stop codons. On the basis of the observation that aminoglycoside treatment can suppress stop codons in cultured cells, we tested the effect of gentamicin on cultured muscle cells from the mdx mouse - an animal model for DMD that possesses a premature stop codon in the dystrophin gene. Exposure of mdx myotubes to gentamicin led to the expression and localization of dystrophin to the cell membrane. We then evaluated the effects of differing dosages of gentamicin on expression and functional protection of the muscles of mdx mice. We identified a treatment regimen that resulted in the presence of dystrophin in the cell membrane in all striated muscles examined and that provided functional protection against muscular injury. To our knowledge, our results are the first to demonstrate that aminoglycosides can suppress stop codons not only in vitro but also in vivo. Furthermore, these results raise the possibility of a novel treatment regimen for muscular dystrophy and other diseases caused by premature stop codon mutations. This treatment could prove effective in up to 15% of patients with DMD.  (+info)

Enhanced expression of recombinant dystrophin following intramuscular injection of Epstein-Barr virus (EBV)-based mini-chromosome vectors in mdx mice. (7/735)

Gene transfer by direct intramuscular injection of naked plasmid DNA has been shown to be a safe, simple but relatively inefficient method for gene delivery in vivo. Eukaryotic plasmid expression vectors incorporating the Epstein-Barr virus (EBV) origin of replication (oriP) and EBNA1 gene have been shown to act as autonomous episomally replicating gene transfer vectors which additionally provide nuclear matrix retention functions. Prolonged expression of a LacZ reporter gene and recombinant human dystrophin was shown using EBV-based plasmid vectors transfected into C2C12 mouse myoblast and myotube cultures. Intramuscular injection of EBV-based dystrophin expression plasmids into nude/mdx mice resulted in significant enhancement in the number of muscle fibres expressing recombinant dystrophin compared with a conventional vector. This effect was observed for over 10 weeks after a single administration. These results indicate the potential advantage of EBV-based expression vectors for focal plasmid-mediated gene augmentation therapy in Duchenne muscular dystrophy (DMD) and a range of other gene therapeutic applications.  (+info)

Local and distant transfection of mdx muscle fibers with dystrophin and LacZ genes delivered in vivo by synthetic microspheres. (8/735)

Patterns of dystrophin and beta-galactosidase expression were examined in mdx mice after i.m. injections of synthetic microspheres (MF-2) loaded with full-length (pHSADy) or mini-dystrophin gene (pSG5dys) cDNA plasmid constructs or with LacZ marker gene (pCMV-LacZ). A single injection of 25 microg pHSADy into quadriceps femoris muscle resulted in 6.8% of dystrophin positive myofibers (DPM) in a given muscle; 8.4% of DPM in glutaeus muscle and 4.3% of DPM in quadriceps femoris muscle of contralateral limb on day 21 after exposure compared with only 0.6% DPM in intact (non-injected) mdx mice. A high proportion of DPM (17.6% and 10.8%, respectively) was registered in both injected and contralateral muscles after mini- gene cDNA administration. MF-2/dystrophin cDNA particles were detected by FISH analysis in about 60-70% of myofiber nuclei in muscles of injected and contralateral limbs 7 days after application. The presence of human dystrophin cDNA and its products in all skeletal muscles and in different internal organs was proven by PCR and RT-PCR analysis. Patches of beta-galactosidase expression were abundant in injected muscle, and frequent in the contralateral and other skeletal muscles as well as in diaphragm, heart and lungs. High levels of dystrophin cDNA expression, and an efficient distant transfection effect with preferential intranuclei inclusion of MF-2 vehicle, are very encouraging for the development of a new constructive strategy in gene therapy trials of DMD.  (+info)

1.Duchenne and Becker muscular dystrophies are X-linked disorders caused by defects in muscle dystrophin. The mdx mouse is an animal model for Duchenne muscular dystrophy which has a point mutation in the dystrophin gene, resulting in little (, 3%) or no expression of dystrophin in muscle. Mdx mice show a characteristic pattern of muscle necrosis and regeneration. Muscles are normal until the third postnatal week when widespread necrosis commences. This is followed by muscle regeneration, with the persistence of centrally nucleated fibres.. 2.This work has examined the hypothesis that the onset of this muscle necrosis is associated with postnatal maturation of the thyroid endocrine system and that pharmacological inhibition of thyroid hormone synthesis delays the onset of muscle necrosis.. 3.Serum T4 and T3 concentrations of mice were found to rise immediately before the onset of muscle necrosis in the mdx mouse, and induction of hypothyroidism by treatment of animals with propylthiouracil was ...
Duchenne muscular dystrophy (DMD) is a fatal neuromuscular disorder caused by mutations in the Dmd gene. In addition to skeletal muscle wasting, DMD patients develop cardiomyopathy, which significantly contributes to mortality. Antisense oligonucleotides (AOs) are a promising DMD therapy, restoring functional dystrophin protein by exon skipping. However, a major limitation with current AOs is the absence of dystrophin correction in heart. Pip peptide-AOs demonstrate high activity in cardiac muscle. To determine their therapeutic value, dystrophic mdx mice were subject to forced exercise to model the DMD cardiac phenotype. Repeated peptide-AO treatments resulted in high levels of cardiac dystrophin protein, which prevented the exercised induced progression of cardiomyopathy, normalising heart size as well as stabilising other cardiac parameters. Treated mice also exhibited significantly reduced cardiac fibrosis and improved sarcolemmal integrity. This work demonstrates that high levels of cardiac
TY - JOUR. T1 - Loss of peroxiredoxin-2 exacerbates eccentric contraction-induced force loss in dystrophin-deficient muscle. AU - Olthoff, John T.. AU - Lindsay, Angus. AU - Abo-Zahrah, Reem. AU - Baltgalvis, Kristen A.. AU - Patrinostro, Xiaobai. AU - Belanto, Joseph J.. AU - Yu, Dae Yeul. AU - Perrin, Benjamin J.. AU - Garry, Daniel J.. AU - Rodney, George G.. AU - Lowe, Dawn A.. AU - Ervasti, James M.. PY - 2018/12/1. Y1 - 2018/12/1. N2 - Force loss in skeletal muscle exposed to eccentric contraction is often attributed to injury. We show that EDL muscles from dystrophin-deficient mdx mice recover 65% of lost force within 120 min of eccentric contraction and exhibit minimal force loss when the interval between contractions is increased from 3 to 30 min. A proteomic screen of mdx muscle identified an 80% reduction in the antioxidant peroxiredoxin-2, likely due to proteolytic degradation following hyperoxidation by NADPH Oxidase 2. Eccentric contraction-induced force loss in mdx muscle was ...
Although the cause of Duchenne muscular dystrophy (DMD) is known, the specific factors that initiate and perpetuate disease progression are not well understood. We hypothesized that leaky dystrophin-deficient skeletal muscle releases endogenous danger signals (TLR ligands), which bind to Toll-like receptors (TLRs) on muscle and immune cells and activate downstream processes that facilitate degeneration and regeneration in dystrophic skeletal muscle. Here, we demonstrate that dystrophin-deficient mouse muscle cells show increased expression of several cell-surface and endosomal TLRs. In vitro screening identified ssRNA as a relevant endogenous TLR7 ligand. TLR7 activation led to myd88-dependent production of pro-inflammatory cytokines in dystrophin-deficient muscle cells, and cause significant degeneration/regeneration in vivo in mdx mouse muscle. Also, knockout of the central TLR adaptor protein, myd88 in mdx mice significantly improved skeletal and cardiac muscle function. Likewise, ...
Dystrophin deficiency sensitizes skeletal muscle of mice to eccentric contraction (ECC)-induced strength loss. ECC protocols distinguish dystrophin-deficient from healthy, wild type muscle, and test the efficacy of therapeutics for Duchenne muscular dystrophy (DMD). However, given the large lab-to-lab variability in ECC-induced strength loss of dystrophin-deficient mouse skeletal muscle (10-95%), mechanical factors of the contraction likely impact the degree of loss. Therefore, the purpose of this study was to evaluate the extent to which mechanical variables impact sensitivity of dystrophin-deficient mouse skeletal muscle to ECC. We completed ex vivo and in vivo muscle preparations of the dystrophin-deficient mdx mouse and designed ECC protocols within physiological ranges of contractile parameters (length change, velocity, contraction duration, and stimulation frequencies). To determine whether these contractile parameters affected known factors associated with ECC-induced strength loss, we measured
1. The functional properties of tibialis anterior muscles of normal adult (C57BL/10) and age-matched dystrophin-deficient (C57BL/10 mdx) mice have been investigated in situ. Comparisons were made between tibialis anterior muscle strength, rates of force development and relaxation, force-frequency responses and fatiguability. Subjecting mdx and C57 muscles to a regimen of eccentric exercise allowed the hypothesis to be tested that dystrophin-deficient muscles are more susceptible to exercise-induced muscle damage.. 2. mdx muscles were, on average, 30% stronger than C57 muscles and almost 80% heavier, but both had similar muscle lengths. Thus, although mdx muscles were stronger in absolute terms, their estimated force per unit cross-sectional area was significantly less than that of C57 muscles.. 3. The force-frequency relationships of C57 and mdx muscles differed in that whilst, at 40 Hz, the former developed 70% of the force developed at 100 Hz, the latter developed only 55% of the maximal ...
Cardiomyocyte calcium overloading has been implicated in the pathogenesis of Duchenne muscular dystrophy (DMD) heart disease. The cardiac isoform of sarcoplasmic reticulum calcium ATPase (SERCA2a) plays a major role in removing cytosolic calcium duri
Fibrosis is a common pathological feature observed in muscle from patients with Duchenne muscular dystrophy (DMD). In the dystrophic (mdx) mouse model of DMD, the diaphragm is more severely affected than other skeletal muscles. The level of transforming growth factor-beta1 (TGF-beta1), an inflammato …
Recordings of single-channel activity were made from cell-attached patches on cerebellar granule cells from normal and mdx mice. Recordings from mdx granule cells show the activity of ion channels that are open for seconds at negative holding potentials near rest. These channels are permeable to divalent cations and have a conductance of 8-10 pS with either Ca2+ or Ba2+ as the charge carrier in the patch electrode. Under similar recording conditions, channel activity is virtually absent from normal mouse granule cells. The absence of dystrophin in neurons, as well as in skeletal muscle, is associated with an increase in the activity of Ca2+-permeable ion channels. Increased channel activity may be an early event leading to pathophysiological accumulation of intracellular Ca2+ in Duchenne muscular dystrophy. ...
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In a previous STSM, I travelled to the UK initiate a collaboration with Professor Wells and his lab at the Royal Veterinary College. There we performed experiments in the mdx dystrophic mice model with the main aim to get a proof of principle of the plasmid based microminicircle (miMC) as a functional vector for splice correction. Analysis two weeks after treatment showed expression of the splice correcting U7snRNA but low levels of rescued protein expression. This is in accordance to the Goyenvalle studies using AAV vectors, where only a minute effect was seen after two weeks, but an accumulation of exon skipped dystrophin protein was seen at later time points.. The aim of this STSM was to return to the Wells group at the RVC to follow up the investigation of the miMC as a vector for exon-skipping. I analyzed tissue from experiments where the mdx mice had been treated with a miMC expressing an exon skipping snRNA four and nine weeks before harvest. Furthermore, mdx mice had also been treated ...
Systemic administration of IGF-I enhances oxidative status and reduces contraction-induced injury in skeletal muscles of mdx dystrophic mice Academic Article ...
Fingerprint Dive into the research topics of Nitric oxide controls fat deposition in dystrophic skeletal muscle by regulating fibro-adipogenic precursor differentiation. Together they form a unique fingerprint. ...
07/21/2014 Kit Components Product code D600B-C Components: D600 Description GoTaq MDx Hot Start Polymerase, 500u GoTaq MDx Hot Start Polymerase Side: 1/6 * 1 Identifikasjon av kjemikaliet og ansvarlig
Whenever I hear the words MD Breakthrough, my heart skips a beat. Is it really? Often, it is in reference to a study in the mdx mouse. We can cure mice. But…
Rebuildable ACURA MDX TECH 2007 with primary damage FRONT END is for sale in GREENWELL SPRINGS LA, Lot 27100975. VIN 2HNYD28357H540462
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Duchenne muscular dystrophy (DMD) is caused by mutations in the DMD gene that abolish the synthesis of dystrophin protein. Antisense oligonucleotides (AOs) targeted to trigger excision of an exon bearing a mutant premature stop codon in the DMD transcript have been shown to skip the mutated exon and partially restore functional dystrophin protein in dystrophin-deficient mdx mice. To fully exploit the therapeutic potential of this method requires highly efficient systemic AO delivery to multiple muscle groups, to modify the disease process and restore muscle function. While systemic delivery of naked AOs in DMD animal models requires high doses and is of relatively poor efficiency, we and others have recently shown that short arginine-rich peptide-AO conjugates can dramatically improve in vivo DMD splice correction. Here we report for the first time that a chimeric fusion peptide (B-MSP-PMO) consisting of a muscle-targeting heptapeptide (MSP) fused to an arginine-rich cell-penetrating peptide (B-peptide)
Duchenne muscular dystrophy is a neuromuscular disease caused by the lack of dystrophin that affects skeletal muscles, causing degeneration of muscle fibers and replacing them with fibrous and adipose tissue, events that gradually lead to functional loss. Patients with Duchenne muscular dystrophy have shown that bones become more fragile with age and with advancement of the disease. Muscle weakness and reduced mobility have been suggested to be the factors that promote bone deterioration. However, it seems that this does not occur in mdx mice. It has been identified in mdx mice the existence of a factor related or not to the lack of dystrophin that also participates in the impairment of bone quality. Mdx mice also exhibit muscle degeneration, but unlike human, it is compensated by muscle regeneration. In consequence, there is an increase in the muscle mass, but not necessarily of muscle contractile strength. The accommodation of this increased muscle mass promotes bone formation at specific ...
As a target for gene therapy, Duchenne muscular dystrophy (DMD) presents many obstacles but also an unparalleled prospect for correction by alternative splicing. The majority of mutations in the dystrophin gene occur in the region encoding the spectrin-like central rod domain, which is largely dispensable. Thus, splicing around mutations can generate a shortened but in-frame transcript, permitting translation of a partially functional dystrophin protein. We have tested this idea in vivo in the mdx dystrophic mouse (carrying a mutation in exon 23 of the dystrophin gene) by combining a potent transfection protocol with a 2-O-methylated phosphorothioated antisense oligoribonucleotide (2OMeAO) designed to promote skipping of the mutated exon*. The treated mice show persistent production of dystrophin at normal levels in large numbers of muscle fibers and show functional improvement of the treated muscle. Repeated administration enhances dystrophin expression without eliciting immune responses. Our ...
Patterns of dystrophin and β-galactosidase expression were examined in mdx mice after i.m. injections of synthetic microspheres (MF-2) loaded with full-length (pHSADy) or mini-dystrophin gene (pSG5dys) cDNA plasmid constructs or with LacZ marker gene (pCMV-LacZ). A single injection of 25 μg pHSADy into quadriceps femoris muscle resulted in 6.8% of dystrophin positive myofibers (DPM) in a given muscle; 8.4% of DPM in glutaeus muscle and 4.3% of DPM in quadriceps femoris muscle of contralateral limb on day 21 after exposure compared with only 0.6% DPM in intact (non-injected) mdx mice. A high proportion of DPM (17.6% and 10.8%, respectively) was registered in both injected and contralateral muscles after mini- gene cDNA administration. MF-2/dystrophin cDNA par- ticles were detected by FISH analysis in about 60-70% of myofiber nuclei in muscles of injected and contralateral limbs 7 days after application. The presence of human dystrophin cDNA and its products in all skeletal muscles and in different
Extracellular microRNAs (miRNAs) are promising biomarkers of the inherited muscle wasting condition Duchenne muscular dystrophy, as they allow non-invasive monitoring of either disease progression or response to therapy. In this study, serum miRNA profiling reveals a distinct extracellular miRNA signature in dystrophin-deficient mdx mice, which shows profound dose-responsive restoration following dystrophin rescue. Extracellular dystrophy-associated miRNAs (dystromiRs) show dynamic patterns of expression that mirror the progression of muscle pathology in mdx mice. Expression of the myogenic miRNA, miR-206 and the myogenic transcription factor myogenin in the tibialis anterior muscle were found to positively correlate with serum dystromiR levels, suggesting that extracellular miRNAs are indicators of the regenerative status of the musculature. Similarly, extracellular dystromiRs were elevated following experimentally-induced skeletal muscle injury and regeneration in non-dystrophic mice. Only a minority
TY - JOUR. T1 - Muscle-bone interactions in dystrophin-deficient and myostatin-deficient mice. AU - Montgomery, Eric. AU - Pennington, Catherine. AU - Isales, Carlos M.. AU - Hamrick, Mark W.. PY - 2005/9/1. Y1 - 2005/9/1. N2 - We have investigated muscle-bone interactions using two mouse mutants that are known to differ from normal mice in skeletal muscle growth and development: mice lacking myostatin (GDF8) and mice lacking dystrophin (mdx). Myostatin-deficient mice show increased muscle size and strength compared to normal mice, whereas the mdx mouse is a well-established animal model for Duchenne muscular dystrophy. The mdx mice have significantly larger hindlimb muscles than controls, and histological sections of the quadriceps muscles show dystrophic changes with extensive fibrosis. Femoral bone mineral density (BMD) and fracture strength (Fu) are significantly greater in mdx mice than controls, and these variables are more strongly correlated with quadriceps muscle mass than with body ...
The requirement of muscle damage for effective delivery and AON induced dystro-phin expression is further supported by the result obtained in the cardiac muscle in the mdx mice after systemic treatment of both 2OMePS AON and PMO. Cardiac muscles in mdx mice are less affected by the dystrophic process and no significant pathology and functional impairment can be obviously demonstrated until late age. Consistently, only minimum amount of dystrophin expression can be detected in the cardiac muscle even after repeated injections of both 2OMePS AON and PMO in all mdx mice aged 6 months or younger (Lu et al. 2005; Alter et al. 2006), whereas the same treatment can induce high levels of dystrophin in skeletal muscles. There is a possibility that the special tissue structure (vasculature and membrane) and pattern of metabolism or gene expression regulation could be responsible for lower efficiency of AON delivery or exon skipping inside myonuclei. However, direct injection of AON into cardiac muscles ...
The identification of the channel involved in store-dependent Ca entry has been largely investigated in nonexcitable cells. Candidate molecules for this pathway probably belong to the TRPC protein family (for review see Clapham et al., 2001). This has been shown by heterologous expression of TRPC proteins. Using this methodology, TRPC1-5 have been shown to be possibly activated by store depletion (Groschner et al., 1998; Philipp et al., 1998; Vannier et al., 1999; Warnat et al., 1999; Liu et al., 2000). However, other authors showed that heterologous expression of TRPC3-6 also gave channels whose activation was independent of store depletion (Zitt et al., 1997; Hofmann et al., 1999; Schaefer et al., 2000; Wu et al., 2000). Therefore, different groups tried to evaluate the functional role of TRPC proteins by repressing their expression by transfection with antisense nucleotide sequences. This strategy has been used to clearly demonstrate that TRPC4 is part of the native Ca-release activated Ca ...
Introduction: In Duchenne muscular dystrophy and in the mdx mouse, muscle fiber degeneration and subsequent fibrosis lead to cardiorespiratory failure. Previously, we demonstrated that the anti-fibrotic agent suramin was effective in decreasing fibrosis in mdx muscles. In this study, we were interested to see whether suramin could affect metalloproteinases (MMP) and improve the functional activity of the mdx diaphragm muscle. Methods: Zymography was performed to evaluate MMP-2 and MMP-9 activity. Western blotting was used to analyze the levels of beta-dystroglycan. Muscle function was assessed in hemidiaphragm in vitro preparations. Results: We found that suramin affects metalloproteinase-9 activity and increases beta-dystroglycan. Furthermore, suramin also protects against diaphragm muscle fatigue over time. Conclusions: These results show the potential benefits of suramin in maintaining the structure of the dystrophin-glycoprotein complex. Muscle Nerve, 46:810813, ...
Figure 2: Histopathology related to injury. (a) cross-section of normal healthy TA muscle from a wild-type mouse. Skeletal muscle fibers are multinucleated and the nuclei stain blue; the sarcoplasm of each cell stains pink. (b) cross-section from a wild-type TA after injury. There was only minimal evidence of perivascular inflammation (arrows) in the wild-type tissue after injury. (c) cross-section from TA muscle of an mdx mouse. Even without injury, there is mild inflammation, slight increase in endomysial connective tissue, heterogeneity in fiber size, and many centrally nucleated fibers (CNFs, open arrows), all indicative of ongoing degeneration/regeneration within the muscle. (d) cross-section from an mdx TA after injury. Even with a protocol that produces mild changes in morphology to healthy muscle, the mdx muscle suffers much more damage, such as myonecrosis, myophagocytosis, and foci of inflammation surrounding individual muscle fibers (closed arrows). Scale bar = 40 μm ...
PubMed journal article: Myostatin genetic inactivation inhibits myogenesis by muscle-derived stem cells in vitro but not when implanted in the mdx mouse muscle. Download Prime PubMed App to iPhone, iPad, or Android
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Lack of dystrofin is associated with altered integration of the mitochondria and ATPases in slow-twitch muscle cells of MDX mice (kaasautor). // Biohim. Biophys. Acta 1505 (2001) 2-3: Butadioon-monoksiimi toime mitokondrite ja ATPaaside integratsioonile südamelihases (kaasautor). // Eesti Arst (2003) ...
MDX injection is one of the most threats for application written for Web. Big data is the ability of providing exponential growth and ava...
This is a multicenter, follow-up study in up to 191 subjects with metastatic melanoma who were previously enrolled and treated in ipilimumab studies MDX010-02, MDX010-08, and MDX010-15. The purpose of this study is to 1) collect the date and cause of death, if known, for all deceased subjects who participated in any of the specified studies; 2) collect the date of progression for subjects who completed the studies with stable disease or better; and 3) prospectively follow all surviving subjects to determine progression-free and overall survival ...
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As more fully set forth in this websites terms of use. (1) nothing contained on or offered by or through this website should be construed as medical advice and should not be relied upon for medical diagnosis or treatment. MDX Medical, Inc. (MDX). the provider of this website, does not recommend or endorse any particular healthcare provider whose information or ratings appear on this website; and (2) MDX has granted you a limited license to access and use this website for your own noncommercial use. You are not permitted to copy, reproduce, distribute, transmit, mirror, frame, scrape, extract, wrap, create derivative works of, reverse engineer, decompile or disassemble any part or aspect of this website. ...
Duchenne muscular dystrophy (DMD) is a fatal genetic disease caused by mutations in the dystrophin gene. Lack of dystrophin causes muscle wasting that eventually leads to premature death from respiratory and/or heart failure. Heart disease can be detected as early as six years of age in DMD patients. As heart disease progresses, patients begin to show overt signs of severe dilated cardiomyopathy and up to 40% of patients die from heart disease. Adeno-associated virus (AAV) mediated micro-dystrophin gene therapy brings the hope of ameliorating DMD. Dystrophin-deficient mdx mice exhibit a cardiac phenotype similar to human DMD patients albeit at a delayed progression (Bostick et al 2008 Cir Res102:121-130). Delineating the potential of gene therapy at different stages of dystrophic cardiomyopathy is critical to developing a successful gene therapy strategy. Investigators, including ourselves, have previously shown that AAV-9 can efficiently transduce the adult mouse heart. In this study, we ...
Duchenne muscular dystrophy (DMD), a recessive X-linked disorder and the most common of a class of progressive muscle-wasting diseases, is characterized by the lack of dystrophin at the muscle cell membrane (1). Replacement of absent dystrophin in mdx mice by transgenic expression leads to complete restoration of normal muscle cell membrane function (2). Muscle is known to be a regenerative tissue, and this regeneration is accomplished via a heterogeneous population of cells called satellite cells or myoblasts. These cells divide upon damage to muscle, fuse to one another and with existing myofibers, and create new muscle fibers. One approach to therapy for DMD was the intramuscular injection of normal myoblasts into the skeletal muscle of DMD patients or mdx mice, which lack full-length functional dystrophin. Although early results in mdx mice were promising, the human clinical trials proved safe but ineffective, with little or no new expression of dystrophin documented (3, 4). Originally, it ...
Duchenne Muscular Dystrophy (DMD) is a progressive lethal disease caused by X-linked mutations of the dystrophin gene. Dystrophin deficiency clinically man
Researchers describe how increased production of a microRNA promotes progressive muscle deterioration in a mouse model of Duchenne muscular dystrophy (DMD), according to a study published online on January 2, 2012 in the Journal of Cell Biology. As DMD patients age, their damaged muscle cells are gradually replaced by collagen-rich, fibrous tissue. This muscle fibrosis is partly induced by the growth factor TGF-beta, which is highly activated in DMD patients, though exactly how this cytokine promotes fibrogenesis is unclear. Dr. Pura Muñoz-Cánoves and colleagues examined the role of miR-21, a microRNA whose production is stimulated by TGF-beta signaling. miR-21 was upregulated in the collagen-producing fibroblasts of both DMD patients and mice that develop disease symptoms similar to human muscular dystrophy (so-called mdx mice). Inhibiting miR-21 reduced collagen levels and prevented, or even reversed, fibrogenesis in diseased mice, whereas mdx mice overexpressing the microRNA produced more ...
SingleCut CRISPR efficiently restored up to 90% of dystrophin expression in skeletal and heart muscles. Study published in the journal Science Translational Medicine. CAMBRIDGE, Mass. - November 29, 2017 - Exonics Therapeutics, Inc., a biotechnology company focused on developing SingleCut CRISPR technology to repair mutations causing Duchenne muscular dystrophy and other neuromuscular diseases, today announced the publication of a preclinical study demonstrating the Companys SingleCut CRISPR technology efficiently corrected in vivo dystrophin expression in a mouse model of Duchenne.. Duchenne is a devastating muscle disease in children for which there is no cure. It is caused by mutations of the dystrophin protein gene responsible for stabilizing and protecting muscle fibers, which results in progressive muscle weakness and leads to life-threatening and ultimately fatal medical issues. Data suggest that deletions of exon 50 of the dystrophin gene are among the most common single exon deletions ...
Induced splice modulation of pre-mRNAs shows promise to correct aberrant disease transcripts and restore functional protein and thus has therapeutic potential. Duchenne muscular dystrophy (DMD) results from mutations that disrupt the DMD gene open reading frame causing an absence of dystrophin protein. Antisense oligonucleotide (AO)-mediated exon skipping has been shown to restore functional dystrophin in mdx mice and DMD patients treated intramuscularly in two recent phase 1 clinical trials. Critical to the therapeutic success of AO-based treatment will be the ability to deliver AOs systemically to all affected tissues including the heart. Here, we report identification of a series of transduction peptides (Pip5) as AO conjugates for enhanced systemic and particularly cardiac delivery. One of the lead peptide-AO conjugates, Pip5e-AO, showed highly efficient exon skipping and dystrophin production in mdx mice with complete correction of the aberrant DMD transcript in heart, leading to |50% of the normal
The investigators previous work in males with Becker Muscular Dystrophy shows that functional sympatholysis is restored by acute inorganic nitrate supplementation. This was translated from work using the mdx mouse model of dystrophinopathy. Recent work has shown that there is a frank improvement in grip strength when mdx mice are treated with an inorganic Nitric Oxide (NO) donor. The purpose of this study is to determine if chronic treatment with an inorganic NO donor can benefit patients with muscular dystrophy beyond blood flow regulation ...
Duchenne muscular dystrophy (DMD) is a neuromuscular disorder causing progressive muscle degeneration. Although cardiomyopathy is a leading mortality cause in DMD patients, the mechanisms underlying heart failure are not well understood. Previously, we showed that NF-κB exacerbates DMD skeletal muscle pathology by promoting inflammation and impairing new muscle growth. Here, we show that NF-κB is activated in murine dystrophic (mdx) hearts, and that cardiomyocyte ablation of NF-κB rescues cardiac function. This physiological improvement is associated with a signature of upregulated calcium genes, coinciding with global enrichment of permissive H3K27 acetylation chromatin marks and depletion of the transcriptional repressors CCCTC-binding factor, SIN3 transcription regulator family member A, and histone deacetylase 1. In this respect, in DMD hearts, NF-κB acts differently from its established role as a transcriptional activator, instead promoting global changes in the chromatin landscape to regulate
The cytotoxic T cell (CT) GalNAc transferase (Galgt2) is confined to the NMJ and catalyzes addition of the terminal β1,4 GalNAc residues onto the CT carbohydrate of a subset of α-DG proteins [71, 72]. α-DG is the predominant glycoprotein modified with the CT carbohydrate in skeletal muscle where it is enriched at the postsynaptic membrane of the NMJ [73]. Overexpression of Galgt2 in mdx mice increases abundance and extrasynaptic expression of α-DG modified with the CT antigen, resulting in improved laminin-binding activity [72, 73]. Overexpression of SSPN in mdx mice increases GalNAc modifications in a similar manner to the overexpression of Galgt2, as revealed by the increased cell surface binding of the lectin Wisteria floribunda agglutinin (WFA) [19], which is a marker for NMJ-specific CT carbohydrate modification of α-DG (Figure 2) [72, 74-77]. WFA binding is localized to NMJs in normal muscle and is increased around the extra-synaptic sarcolemma of mdx muscle cryosections [19, 78]. WFA ...
Bouts of intrinsic myonecrosis in DMD can also directly damage neuromuscular junctions (NMJs; Box 1). The adverse progressive changes in NMJs, which indicate denervation, are widely reported in dystrophic muscles of rodent and dog models of DMD (Haddix et al., 2018). These altered NMJs affect the associated dystrophic nerve over time, with consequent increased levels of S100 and Tau5 proteins seen by 13 months of age in sciatic nerves of mdx mice (Gordish-Dressman et al., 2018). Such neuronal changes indicate progressive irreversible denervation, often associated with neurodegeneration (Krishnan et al., 2016), that is likely to become pronounced over many years or decades and contribute to the loss of muscle function in DMD patients. These preclinical neuronal changes could prove useful as a biomarker for the long-term consequences of repeated intrinsic myonecrosis in animal studies.. A key aim for DMD therapies is to prevent myonecrosis and to directly stabilise the myofibres, ideally by ...
Sarepta is preparing to file a follow-up to its Duchenne muscular dystrophy (DMD) treatment Exondys 51 that seems to have greater efficacy.. Shares in the company were on the rise after it reported positive data from a phase I/II trial of the follow-up - called golodirsen - which showed the exon-skipping drug met its primary objective of raising levels of functional dystrophin, a protein that is mutated in DMD leading to the characteristic muscle wasting symptoms.. The European trial in 25 boys with deletions in the dystrophin gene amenable to treatment with golodirsen - an exon 53-skipping drug - showed that dystrophin levels increased to 1.019% of normal from an average baseline level of 0.095% of normal, which is a 10-fold increase and superior to that seen with exon 51-targeting Exondys 51 (eteplirsen). Some clinicians have suggested however that restoring dystrophin levels to 10% may be needed to have a real clinical benefit.. Nevertheless, Sarepta is hoping that data may be enough for it ...
Different strategies involving stem cells for muscular dystrophy may be on the horizon, research suggests. Scientists have been using stem cells isolated from muscle tissue, bone marrow and blood vessels in lab animals to regenerate muscle fibers that are deficient in dystrophin[3] and results are encouraging.. In 2006, researchers managed to restore mobility in two afflicted dogs using stem cells isolated from muscle blood vessels [4], and in 2007 scientists managed to treat Duchenne MD in research mice using a combination of genetic correction and stem cells [3]. The latter study showed that it is possible to correct the genetic error in the cells that no longer produce dystrophin protein, and inject corrected cells stimulating the regeneration of muscles.. Researchers at the Harvard Stem Cell Institute obtained similar results, demonstrating that transplanted muscle stem cells can improve function in mice with MD, while replenishing the stem cell population in muscle fibers [5].. Although ...
A promising minidystrophin gene that restores normal muscle force to skeletal and diaphragm muscles in mice with a disease resembling Duchenne muscular dystrophy (DMD) seems to be only partially effective at restoring strength and function to heart muscles. ...
Soudon reported results for 20+ DMD patients who used CTMV (DMD3). The patients had an average survival of 3.6 years.. Eagle et al. reported results for 200 DMD patients who were either untreated or used TMV/CTMV (DMD4). The untreated patients had an average survival of 19.5 years (cardiomyopathy was the cause of death for 7.4%). The patients who used TMV/CTMV had an average survival of 24.8 years (cardiomyopathy was the cause of death for 36.8%).. Bach et al. reported results for seven DMD patients who used CTMV for a mean of 7.1 years starting from the average age of 21.1±3.8 to 28.1±4.5 years (JBCV7). Two of the seven patients were still alive at time of publication. Complications were not reported.. The DMD patients of Baydur and Bach, who survived long enough after episodes of acute respiratory failure to be referred to rehabilitation centers and who used TMV/CTMV, had an improved average survival of 6.2 and 7.1 years, respectively.. Ishikawa et al. reported that (YICV7):. ...
In a mouse model of dystrophin-deficient cardiomyopathy, ACE-I produced haemodynamic benefit, whereas steroids accelerated progression of cardiomyopathy. Although mouse models may not entirely replicate the human condition, comprehensive monitoring of cardiac function with these therapies is essenti …
Real progress is being made in supplying functional dystrophin genes to treat Duchenne muscular dystrophy (DMD), a disease in which mutated dystrophin genes keep this critical protein from being produced in muscle fibers. ...
S. Fletcher, Honeyman, K., Fall, A. M., Harding, P. L., Johnsen, R. D., Steinhaus, J. P., Moulton, H. M., Iversen, P. L., and Wilton, S. D., Morpholino oligomer-mediated exon skipping averts the onset of dystrophic pathology in the mdx mouse., Molecular therapy : the journal of the American Society of Gene Therapy, vol. 15, no. 9, pp. 1587-92, 2007. ...
S. Fletcher, Honeyman, K., Fall, A. M., Harding, P. L., Johnsen, R. D., Steinhaus, J. P., Moulton, H. M., Iversen, P. L., and Wilton, S. D., Morpholino oligomer-mediated exon skipping averts the onset of dystrophic pathology in the mdx mouse., Molecular therapy : the journal of the American Society of Gene Therapy, vol. 15, no. 9, pp. 1587-92, 2007. ...
Animal models: The consensus is that regulatory toxicology studies should be performed in rodents, whereas other preclinical animal studies may be performed in other animal models. A summary of the relevance of different models (mdx mouse, grmd dog, non-human primate and pig) was provided.. Proof of concept/functionality data/evaluation of oncogenicity: Although proof of concept has to be based on the detection of a functional improvement in the animal model, this needs to be evaluated in the context of the drug in question. In some cases gene expression may be adequate for proof of concept, but functional improvement will have to be seen in a subsequent step. Questions over the percentage of dystrophin expression required, the lack of criteria for the evaluation of efficacy, and how to define the length of follow-up required to prove absence of oncogenicity were also discussed. Route of administration (intramuscular, intravenous, loco-regional) - choice / immune reactions: Overall, the choice ...
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Qiagen has entered into exclusive negotiations to acquire 47 percent of Ipsogens stock. After the completion of that deal, Qiagen would move to acquire all remaining shares of the French cancer molecular diagnostic firm.
JRS: gotta take the tech pkg. mdx handles really nice. u can get a great deal now on suvs. smaller cars are like trending like crazy.. Yeah, its...
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... mice, inbred mdx MeSH B01.050.157.520.440 - mice, inbred cba MeSH B01.050.157.520.445 - mice, inbred cftr MeSH B01.050.157.520. ... mice, inbred mdx MeSH B01.050.199.520.520.440 - mice, inbred cba MeSH B01.050.199.520.520.445 - mice, inbred cftr MeSH B01.050. ... mice, inbred a MeSH B01.050.157.520.318 - mice, inbred akr MeSH B01.050.157.520.338 - mice, inbred balb c MeSH B01.050.157.520. ... mice, inbred a MeSH B01.050.199.520.520.318 - mice, inbred akr MeSH B01.050.199.520.520.338 - mice, inbred balb c MeSH B01.050. ...
Psychiatry related information on Mice, Inbred mdx. *We use dystrophin-deficient mice (mdx), an animal model of DMD, to ... High impact information on Mice, Inbred mdx. *Dystrophin-deficient mice (mdx) expressing a truncated (trc) utrophin transgene ... Anatomical context of Mice, Inbred mdx. *Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse ... Chemical compound and disease context of Mice, Inbred mdx. *C57BL/10SNJ (control) and dystrophic (mdx) mice were given ...
Mesh term Mice, Inbred mdx. Browse to parent terms:. Mice, Inbred C57BL. Mice, Mutant Strains. Description. A strain of mice ... arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. This mutation is X chromosome-linked and produces viable ... The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR ...
Mice, Inbred mdx * Muscular Dystrophy, Duchenne / genetics* * Muscular Dystrophy, Duchenne / metabolism * Nonsense Mediated ... Chromatin immunoprecipitation in muscle showed increased levels of the repressive histone mark H3K9me3 in mdx mice compared to ... wild-type mice, indicating a chromatin conformation less prone to transcription in mdx mice. In line with this finding, ... with the histone deacetylase inhibitor givinostat caused a significant increase in DMD transcript expression in mdx mice. ...
... fundamental principles and the testing of therapeutic hypotheses for treatment of human disease is commonly performed on mouse ... The mdx mouse model as a surrogate for Duchenne muscular dystrophy FEBS J. 2013 Sep;280(17):4177-86. doi: 10.1111/febs.12267. ... Mice, Inbred mdx * Mice, Knockout * Muscular Dystrophy, Duchenne* Substances * Biomarkers Grant support * R01 NS029525/NS/NINDS ... to identify some of the principal classes of obstacles to translation of data from mouse to humans. Of these, the difference in ...
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 [29]. At this ...
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 ... It includes virulence studies in animal fetuses in utero, mouse convulsion bioassay of insulin, quantitation of tumor-initiator ... systems in mouse skin, calculation of potentiating effects of a hormonal factor in an isolated strip of contracting stomach ...
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 ...
Mdx mice, which have the same dystrophin mutation as human patients, are of limited use, as they do not develop early dilated ... Here we summarize the usefulness of the various commonly used DMD mouse models, highlight a model with shortened telomeres like ... Genetic variation in activity of the enzymes of glycolysis and gluconeogenesis between inbred strains of mice. Genetics 89, 551 ... 109 which is not the case for mice in which the mdx mutation was bred to different mouse backgrounds.61 mdx4cv/mTRG2 mice show ...
Massett, M. P., Berk, B. C. Strain-dependent differences in responses to exercise training in inbred and hybrid mice. Am. J. ... mice. Over time, mdx mice hang less long than wild type mice. B. Rotarod running times did not differ between young male mdx (n ... in mdx mice, while they remained low in wild type mice. C-D. Muscles of mdx mice are very vulnerable to treadmill exercise, ... mdx mice are weaker than wild type mice. We do not have data yet on fatigability in older mdx and wild type mice. ...
... reduces necrosis of dystrophic muscle in mdx mice, Neurobiology of Disease" on DeepDyve, the largest online rental service for ... Genetic variability in forced and voluntary endurance exercise performance in seven inbred mouse strains ... Muscle fibers of mdx mice are more vulnerable to exercise than those of normal mice ... Cromolyn administration (to block mast cell degranulation) reduces necrosis of dystrophic muscle in mdx mice. Radley, Hannah G. ...
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 ...
Inbred mdx Mouse NRF2-mediated Notch pathway activation enhances hematopoietic reconstitution following myelosuppressive ... Nrf2 is required for normal postnatal bone acquisition in mice. Kim, J. H., Singhal, V., Biswal, S., Thimmulappa, R. K. & ... Exposure to electronic cigarettes impairs pulmonary anti-bacterial and anti-viral defenses in a mouse model. Sussan, T. E., ...
NRG mutant mice are an immune-deficient irradiation resistant model of Duchenne muscular dystrophy (DMD) for transplantation ... JAX® Mice, Products & Services Conditions of Use. "MICE" means mouse strains, their progeny derived by inbreeding or ... Also Known As: mdx4Cv/NRG. These mdx4Cv/NRG mutant mice are an immune-deficient irradiation resistant model of Duchenne ... These mdx4Cv/NRG mice are homozygous for the Rag1null mutation, and homozygous (for females; or hemizygous for males) for the X ...
... mdx mice [135]. Fukada et al. report that C57BL/6 strain has the best self-renewal capacity among four inbred strains of mdx ... mdx/mTRKO Features of mdx/mTRKO mice: mdx/mTRKO was generated by crossing mdx4cv mice with mice containing deletion in the RNA ... DBA/2-mdx, mdx/mTRKO and dko mouse models provide a more severe dystrophic phenotype than mdx. Mdxβgeo and Dmd-null mice lack ... Mdx mice on C57BL/6 background. Features of C57BL/6-mdx mice: M dx on C57BL/6 background (C57BL/6-mdx) is a novel murine model ...
Inbred mdx Mouse * Evans Blue * Population Biological Variation * Genetic Loci * Loss of Function Mutation ...
... mice, inbred mdx MeSH B01.050.157.520.440 - mice, inbred cba MeSH B01.050.157.520.445 - mice, inbred cftr MeSH B01.050.157.520. ... mice, inbred mdx MeSH B01.050.199.520.520.440 - mice, inbred cba MeSH B01.050.199.520.520.445 - mice, inbred cftr MeSH B01.050. ... mice, inbred a MeSH B01.050.157.520.318 - mice, inbred akr MeSH B01.050.157.520.338 - mice, inbred balb c MeSH B01.050.157.520. ... mice, inbred a MeSH B01.050.199.520.520.318 - mice, inbred akr MeSH B01.050.199.520.520.338 - mice, inbred balb c MeSH B01.050. ...
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 ...
Digit ratio (2Dratio4D) differences between 20 strains of inbred mice. Reginia H Y Yan, Mark Bunning, Douglas Wahlsten, Peter L ... and coordination in the mdx mouse model for DMD. Both invasive and noninvasive tests are available. Tests that do not ... We have previously shown that there is no sex difference in the digit ratios of inbred mice, but found behavioral evidence to ... Here, we examine the fore- and hindlimb digit ratios of 20 inbred mouse strains. We find large inter-strain differences, but no ...
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature. 1992 Jan 1;360(6404 ... Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. / Matsumura, Kiichiro; ... title = "Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle", ... T1 - Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle ...
Glycogen storage disease type III: A novel Agl knockout mouse model. Pagliarani, S., Lucchiari, S., Ulzi, G., Violano, R., ... Glucose-free/high-protein diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice. ... USP8, a regulator of endosomal sorting, is involved in mouse acrosome biogenesis through interaction with the spermatid ESCRT-0 ... Elucidating the role of Agl in bladder carcinogenesis by generation and characterization of genetically engineered mice. ...
Inbred mdx Mouse * Dystrophin * Muscle Strength * Transgenes * Genetic Recombination * Protein-Tyrosine Kinases ...
Inbred mdx Mouse Generation of induced pluripotent stem cells from a Becker muscular dystrophy patient carrying a deletion of ... Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by ... Fibrosis Rescue Improves Cardiac Function in Dystrophin-Deficient Mice and Duchenne Patient-Specific Cardiomyocytes by ...
Disease course in mdx:Utrophin+/− mice: comparison of three mouse models of duchenne muscular dystrophy. McDonald, A. A., ... Sparing of the extraocular muscles in mdx mice with absent or reduced utrophin expression: A life span analysis. McDonald, A. A ... Dystrophic changes in extraocular muscles after gamma irradiation in mdx:utrophin+/- mice. McDonald, A. A., Kunz, M. D. & ... Focusing on fibrosis: Halofuginone-induced functional improvement in the mdx mouse model of Duchenne muscular dystrophy. McLoon ...
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. ...
Dystrophin deficiency in DMD/BMD patients and in the mdx mouse model appears to impair intracellular calcium homeostasis and to ... Dystrophin deficiency in DMD/BMD patients and in the mdx mouse model appears to impair intracellular calcium homeostasis and to ... Dystrophin deficiency in DMD/BMD patients and in the mdx mouse model appears to impair intracellular calcium homeostasis and to ... Dystrophin deficiency in DMD/BMD patients and in the mdx mouse model appears to impair intracellular calcium homeostasis and to ...
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. [ ...
Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Motor Activity, Muscle, Skeletal, Myostatin, Oxidation-Reduction, Physical ... Mice,Mice, Inbred C57BL,Mice, Inbred mdx,Motor Activity,Muscle, Skeletal,Myostatin,Oxidation-Reduction,Physical Conditioning, ... Modestly dystrophic mdx mice were injected with sActRIIB-Fc or PBS with or without voluntary wheel running exercise for 7 wk. ... Modestly dystrophic mdx mice were injected with sActRIIB-Fc or PBS with or without voluntary wheel running exercise for 7 wk. ...
... we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mdx mice lacking ... we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mdx mice lacking ... we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mdx mice lacking ... we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. Mdx mice lacking ...
Inbred mdx Mouse Medicine & Life Sciences * Muscular Dystrophies Medicine & Life Sciences View full fingerprint ... Methods: DMD patient biopsies and samples from mdx mice and golden retriever muscular dystrophy dog samples (with appropriate ... Methods: DMD patient biopsies and samples from mdx mice and golden retriever muscular dystrophy dog samples (with appropriate ... Methods: DMD patient biopsies and samples from mdx mice and golden retriever muscular dystrophy dog samples (with appropriate ...
  • The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR DYSTROPHY. (mcw.edu)
  • These mdx 4Cv /NRG mutant mice are an immune-deficient irradiation resistant model of Duchenne muscular dystrophy (DMD) for transplantation experiments with human cells, such as human induced pluripotent stem cells (hiPSC). (jax.org)
  • The absence of dystrophin leads to a dramatic reduction of the dystrophin-associated proteins (156DAG, 59DAP, 50DAG, 43DAG and 35DAG) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice 2,6-8 . (umn.edu)
  • Here we demonstrate that dystrophin-related protein (DRP, utrophin), an autosomal homologue of dystrophin 9,17 , is associated with an identical or antigenically similar complex of sarcolemmal proteins and that DRP and the dystrophin/DRP-associated proteins colocalize to the neuromuscular junction in Duchenne muscular dystrophy and mdx muscle. (umn.edu)
  • To explore the therapeutic potential of targeting myostatin in settings of muscle degeneration, we crossed myostatin null mutant mice with mdx mice, a model for Duchenne and Becker muscular dystrophy. (elsevier.com)
  • Mutation suppression of stop codons, successfully achieved in the mdx mouse using gentamicin, represents an important evolving treatment strategy in Duchenne muscular dystrophy (DMD). (elsevier.com)
  • The mdx mice model is widely used for Duchenne muscular dystrophy (DMD) studies, which is present in a high percentage from newborns human males. (bvsalud.org)
  • Antisense Oligonucleotides (AO) can facilitate dystrophin expression via targeted exon skipping in cultured cells of Duchenne muscular dystrophy (DMD) patients and in the mouse model of DMD (mdx mice). (elsevier.com)
  • Our findings also provide a possible explanation for the lack of kidney pathology in Duchenne muscular dystrophy patients and mice lacking all dystrophin isoforms. (ox.ac.uk)
  • Membrane abnormalities and Ca homeostasis in muscles of the mdx mouse, an animal model of the Duchenne muscular dystrophy: a review. (naver.com)
  • Bi-specific splice-switching PMO oligonucleotides conjugated via a single peptide active in a mouse model of Duchenne muscular dystrophy. (ox.ac.uk)
  • Studies to date have focused on the delivery of a single SSO conjugated to a CPP, but here we describe the conjugation of two phosphorodiamidate morpholino oligonucleotide (PMO) SSOs to a single CPP for simultaneous delivery and pre-mRNA targeting of two separate genes, exon 23 of the Dmd gene and exon 5 of the Acvr2b gene, in a mouse model of Duchenne muscular dystrophy. (ox.ac.uk)
  • In contrast, mdx mice, a dystrophin-deficient animal model for Duchenne muscular dystrophy, showed significant Evans blue accumulation in skeletal muscle fibers. (nih.gov)
  • Exercise metabolism in Duchenne muscular dystrophy: a biochemical and [31P]-nuclear magnetic resonance study of mdx mice. (ox.ac.uk)
  • Intracellular pH, ratios of phosphocreatine (PCr) to ATP and PCr to inorganic phosphate (Pi) as well as isometric tension were measured during 1 Hz sciatic nerve stimulation and during recovery in the calf muscles of mdx (a model of Duchenne muscular dystrophy) and control mice. (ox.ac.uk)
  • Implications for Cardiac Function Following Rescue of the Dystrophic Diaphragm in a Mouse Model of Duchenne Muscular Dystrophy. (ox.ac.uk)
  • ADAM12 is a disintegrin and metalloprotease, previously demonstrated to significantly alleviate the pathology of mdx mice, a model for Duchenne muscular dystrophy in humans. (ox.ac.uk)
  • A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. (mcw.edu)
  • Erratum to: Voluntary resistance wheel exercise from mid-life prevents sarcopenia and increases markers of mitochondrial function and autophagy in muscles of old male and female C57BL/6J mice. (edu.au)
  • Mice, Inbred C57BL" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (uchicago.edu)
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  • Control quadriceps cryosections from wild-type (C57BL/6) or mdx 4cv mice (top). (cdc.gov)
  • Chromatin immunoprecipitation in muscle showed increased levels of the repressive histone mark H3K9me3 in mdx mice compared to wild-type mice, indicating a chromatin conformation less prone to transcription in mdx mice. (nih.gov)
  • This review focuses on the example of skeletal muscle disease, in particular muscular dystrophy, to identify some of the principal classes of obstacles to translation of data from mouse to humans. (nih.gov)
  • In particular, although these models recapitulate the human DMD skeletal muscle phenotype better than the mdx mouse, the cardiac phenotype is not fully recapitulated. (nature.com)
  • Therefore, assessing the effect of potential therapeutic compounds on muscle performance pre clinically in mouse models is of great importance. (jove.com)
  • This research identified high biological variation between individual mdx mice in the severity of the dystrophic pathology, and supported a relationship between extent of muscle damage in adult mdx mice and their individual enthusiasm for voluntary wheel running. (deepdyve.com)
  • The mdx5cv mouse is a model of DMD in which a point mutation in exon 10 of the dystrophin gene creates an artificial splice site.Using primary myoblasts from mdx5cv mice, single-stranded DNA oligonucleotides were designed to correct this DNA mutation.Exogenous manipulations, such as RNAi, are certainly feasible and possibly required to increase the successful application of gene repair in some primary or progenitor muscle cells. (nih.gov)
  • No dystrophin protein was detected in gastrocnemius muscle from 6 month old mice by western blot and immunohistochemical analysis. (jax.org)
  • At 6 months of age, mdx 4Cv /NRG mice exhibit impaired mobility, defective membrane integrity in muscle fibers, as assayed by Evans blue dye staining, and increased collagen deposition in gastrocnemius and hamstring muscles, compared to NRG mice. (jax.org)
  • Histological examination of muscle from mutant mice reveals centronucleated fibers, mononuclear cell infiltration and increased variability in fiber size. (jax.org)
  • The DRP and dystrophin/DRP-associated proteins are found throughout the sarcolemma in small-calibre skeletal muscles and cardiac muscle of adult mdx mice. (umn.edu)
  • Running also enhanced/restored the markers of muscle oxidative capacity and autophagy in mdx mice to or above the levels of. (lu.se)
  • We used clustered regularly interspaced short palindromic repeat/Cas9 (CRISPR/Cas9)-mediated genome editing to correct the dystrophin gene (Dmd) mutation in the germ line of mdx mice, a model for DMD, and then monitored muscle structure and function. (nih.gov)
  • The degree of muscle phenotypic rescue in mosaic mice exceeded the efficiency of gene correction, likely reflecting an advantage of the corrected cells and their contribution to regenerating muscle. (nih.gov)
  • 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 that SFN treatment increased the expression and activity of muscle phase II enzymes NAD(P)H quinone oxidoreductase 1 and heme oxygenase-1 with a Nrf2-dependent manner. (nih.gov)
  • Collectively, these results show that SFN can improve muscle function and pathology and protect dystrophic muscle from oxidative damage in mdx mice associated with Nrf2 signaling pathway, which indicate Nrf2 may have clinical implications for the treatment of patients with muscular dystrophy. (nih.gov)
  • We have developed a double knockout mouse model, which also shows reduced muscle strength, but is protected from stretch-induced eccentric damage with age. (cdc.gov)
  • Muscle necrosis in mdx mice is rare before the 2nd week of life, but becomes pronounced from weeks 2 to 6. (springernature.com)
  • Using sequential microscopic histology, immunohistochemistry and histomorphometry, we studied the evolution of muscle pathology throughout the mdx life span. (springernature.com)
  • Diminishing necrosis rather than increasing regeneration may explain the differences in muscle pathology observed between human DMD and mouse mdx. (springernature.com)
  • This study shows that muscle TCTP and mTOR signaling are upregulated in a range of mouse models (mdx mouse, mechanical load-induced hypertrophy, and denervation- and immobilization-induced atrophy). (edu.au)
  • Expression microarray analysis revealed decreased aerobic metabolism in the gastrocnemius muscle of mdx mice compared to healthy mice. (unboundmedicine.com)
  • In conclusion, exercise alone or in combination with myostatin/activin blocking corrects aerobic gene expression profiles of dystrophic muscle toward healthy wild type mice profiles. (unboundmedicine.com)
  • In this study we show that copolymers of cationic poly(ethylene imine) (PEI) and poly(ethylene glycol) (PEG) facilitated efficient cellular uptake and nuclear delivery of AO in mature skeletal muscle fibers isolated from mdx mice. (elsevier.com)
  • Sirsi, SR , Williams, JH & Lutz, GJ 2005, ' Poly(ethylene imine)-poly(ethylene glycol) copolymers facilitate efficient delivery of antisense oligonucleotides to nuclei of mature muscle cells of mdx mice ', Human Gene Therapy , vol. 16, no. 11, pp. 1307-1317. (elsevier.com)
  • Results show that Spc5-12 promoter, in combination with an AAV serotype that has high tropism for the heart, drives high MD1 expression levels in cardiac muscle in mdx mice. (vub.be)
  • The mdx mouse, however, does not show significant muscle weakness, and the diaphragm muscle is significantly more degenerated than skeletal muscles. (ox.ac.uk)
  • Normal dystrophin expression is lacking in skeletal muscle and the central nervous system (CNS) of both DMD children and the mdx mouse model. (ox.ac.uk)
  • Extracellular dystrophy-associated miRNAs (dystromiRs) show dynamic patterns of expression that mirror the progression of muscle pathology in mdx mice. (ox.ac.uk)
  • Similarly, extracellular dystromiRs were elevated following experimentally-induced skeletal muscle injury and regeneration in non-dystrophic mice. (ox.ac.uk)
  • Evans blue, a low molecular weight diazo dye, does not cross into skeletal muscle fibers in normal mice. (nih.gov)
  • We furthermore assessed Evans blue incorporation in skeletal muscle of the dystrophia muscularis (dy/dy) mouse and its milder allelic variant, the dy2J/dy2J mouse, animal models for congenital muscular dystrophy. (nih.gov)
  • Grouped EBD-positive fibers were only detected in mdx mice (d), whereas cryosections from normal control mice (b), dy/dy (f), or dy2J/dy2J mice did not show dye uptake into groups of muscle fibers. (nih.gov)
  • Because of the increase in connective tissue in dystrophic muscle, the mice showed a blue aspect resulting from dye accumulation into their connective tissue. (nih.gov)
  • Although WT MDSCs are very myogenic in culture and stimulate muscle repair after injury in the aged mdx mouse, myostatin genetic inactivation blocks myotube formation in vitro, but the myogenic capacity is recovered in vivo under the influence of the myostatin+ host-tissue environment, presumably by reactivation of key genes originally silenced in the Mst KO MDSCs. (unboundmedicine.com)
  • TY - JOUR T1 - Myostatin genetic inactivation inhibits myogenesis by muscle-derived stem cells in vitro but not when implanted in the mdx mouse muscle. (unboundmedicine.com)
  • Cellular trafficking determines the exon skipping activity of Pip6a-PMO in mdx skeletal and cardiac muscle cells. (ox.ac.uk)
  • Physiological tests showed that the transgenic mouse muscle functioned in a way similar to normal muscle. (ox.ac.uk)
  • We thus examined the gene expression profile of mdx mouse muscle compared to wild-type mouse muscle and compared the data with that obtained from the transgenic line overexpressing utrophin. (ox.ac.uk)
  • The data confirm that the expression of utrophin in the mdx mouse muscle results in a global gene expression profile more similar to that seen for the wild-type mouse. (ox.ac.uk)
  • Stem cells isolated from human dental pulp and amniotic fluid improve skeletal muscle histopathology in mdx/SCID mice. (pitt.edu)
  • RESULTS: Both populations of cells engrafted within the host muscle of mdx/SCID mice and through a paracrine effect promoted angiogenesis and reduced fibrosis, which eventually led to an improvement of the histopathology of the dystrophic muscle. (pitt.edu)
  • In a transgenic mdx mouse, where utrophin was over expressed in the skeletal muscle and the diaphragm, but not in the heart, we found cardiac function, specifically right and left ventricular ejection fraction as measured using in vivo magnetic resonance imaging, was restored to wild-type levels. (ox.ac.uk)
  • In mdx mice treated with a peptide-conjugated phosphorodiamidate morpholino oligomer (PPMO) that resulted in high levels of dystrophin restoration in the skeletal muscle and the diaphragm only, cardiac function was also restored to wild-type levels. (ox.ac.uk)
  • In dystrophin/utrophin-deficient double-knockout (dKO) mice, a more severely affected animal model of DMD, treatment with a PPMO again produced high levels of dystrophin only in the skeletal muscle and the diaphragm, and once more restored cardiac function to wild-type levels. (ox.ac.uk)
  • Restoration of diaphragm and other respiratory muscle function, irrespective of the method used, was sufficient to prevent cardiomyopathy in dystrophic mice. (ox.ac.uk)
  • The ratio of PCr/(PCr + Pi) was significantly reduced in mdx as against control muscle during exercise and recovery, but the ratio of PCr/ATP and the half-time for PCr recovery were similar in both strains. (ox.ac.uk)
  • The rate of pH recovery is prolonged in mdx muscle and provides strong evidence for a decline in the capacity of dystrophic muscle to extrude proton equivalents. (ox.ac.uk)
  • In humans, cytoskeletal dystrophin and muscle LIM protein (MLP) gene mutations can cause dilated cardiomyopathy, yet these mutations may have different effects in mice, owing to increased accumulation of other, compensatory cytoskeletal proteins. (ox.ac.uk)
  • More specifically ADAM12 appeared to prevent muscle cell necrosis in the mdx mice as evidenced by morphological analysis and by the reduced levels of serum creatine kinase. (ox.ac.uk)
  • In the present study we demonstrated that ADAM12 may compensate for the dystrophin deficiency in mdx mice by increasing the expression and redistribution of several components of the muscle cell-adhesion complexes. (ox.ac.uk)
  • The small changes in gene expression were unexpected, considering the marked improvement of the mdx pathology when ADAM12 is overexpressed, and suggested that significant changes in mdx/ADAM12 muscle might occur post-transcriptionally. (ox.ac.uk)
  • Activation of protein phosphatase-1 isoforms and glycogen synthase kinase-3 beta in muscle from mdx mice. (semanticscholar.org)
  • 8. Banker BQ - Muscular dysgenesis in the mouse (mdg/mdg): I. Ultrastructural study of skeletal and cardiac muscle. (scielo.br)
  • 9. Banker BQ, Denny-Brown D - A study of denervated muscle in normal and dystrophic mice. (scielo.br)
  • Turk R, Sterrenburg E, de Meijer EJ, van Ommen GJB, den Dunnen JT & 't Hoen PAC (2005), Muscle regeneration in dystrophin-deficient mdx mice studied by gene expression profiling, BMC Genomics 6. (universiteitleiden.nl)
  • Boer JM, de Meijer EJ, Mank EM, van Ommen GB & den Dunnen JT (2002), Expression profiling in stably regenerating skeletal muscle of dystrophin-deficient mdx mice, Neuromuscular Disorders 12. (universiteitleiden.nl)
  • Representative cryosections (heart, quadriceps, and diaphragm muscle) from mdx 4cv mice infused with 4 × 10 14 vector genomes per kilogram of AAV6/CK8-microdystrophin (bottom). (cdc.gov)
  • With two exceptions, studies of myoblast transfer in the mouse have involved transplantation of donor myoblasts isolated from adult or neonatal skeletal muscle satellite cells. (edu.au)
  • In this review we discuss the pathology of DMD, describe the major mouse models that have been developed and their molecular basis, and the relevance of these models to the human disease. (nature.com)
  • An awareness of such distinctions is crucial if we are to avoid misjudging the likely applicability to humans of results obtained on mouse models. (nih.gov)
  • Here we summarize the usefulness of the various commonly used DMD mouse models, highlight a model with shortened telomeres like humans, and identify directions that warrant further investigation. (nature.com)
  • Cmah -deficient mdx mice display an accelerated disease onset and severe cardiac phenotype due to differences in glycosylation between humans and mice. (iospress.com)
  • Like humans, mice are mammals, and their bodies undergo many similar processes, such as ageing, and have similar immune responses to infection and disease. (blood.ca)
  • Physiologically, mice are very like humans, albeit around 3,000 times smaller ( Partridge, 2013 ) but with similar basic body functions such as blood cell production (haematopoiesis), digestion, respiration and the cardiovascular system. (blood.ca)
  • Although differences do exist, mice respond similarly to humans when they are sick or undergo treatment. (blood.ca)
  • Mouse models of various human diseases, including immune thrombocytopenia, have been relatively easy to develop, since mouse physiology and metabolism resemble those of humans. (blood.ca)
  • The extent of dye incorporation in transgenic mice correlated with the phenotypic severity of similar dystrophin mutations in humans. (nih.gov)
  • In conclusion, these results demonstrate a novel way to alleviate dystrophin deficiency in mice, and may stimulate the development of new approaches to compensate for dystrophin deficiency in animals and humans. (ox.ac.uk)
  • Lau L, Spain L. Altered aging-related thymic involution in T cell receptor transgenic, MHC-deficient, and CD4-deficient mice. (labome.org)
  • Knott J, Kurokawa M, Fissore R, Schultz R, Williams C. Transgenic RNA interference reveals role for mouse sperm phospholipase Czeta in triggering Ca2+ oscillations during fertilization. (labome.org)
  • We also studied Evans blue dispersion in transgenic mice bearing different dystrophin mutations, and we demonstrated that cytoskeletal and sarcolemmal attachment of dystrophin might be a necessary requirement to prevent serious fiber damage. (nih.gov)
  • First, we analyzed transgenic mice that overexpress ADAM12 and found mild myopathic changes and accelerated regeneration following acute injury. (ox.ac.uk)
  • We then analyzed changes in gene-expression profiles in mdx/ADAM12 transgenic mice compared with their littermate controls and found only a few genes with an expression change greater than 2-fold between mdx/ADAM12 and mdx. (ox.ac.uk)
  • Bremmer-Bout M, Aartsma-Rus A, de Meijer EJ, Kaman WE, Janson AAM, Vossen RHAM, van Ommen GJB, den Dunnen JT & van Deutekom JCT (2004), Targeted exon skipping in transgenic hDMD mice: A model for direct preclinical screening of human-specific antisense oligonucleotides, Molecular Therapy 10(2). (universiteitleiden.nl)
  • Extracellular matrix genes were up-regulated in mdx to levels similar to those in DMD. (elsevier.com)
  • With the development of high-throughput sequencing, researchers were able to perform deep approaches to investigate which genes are affected by inbreeding and reveal some molecular underpinnings of inbreeding depression. (springer.com)
  • The mdx mouse, an animal model with dystrophin mutation, has a milder phenotype. (bvsalud.org)
  • Together, these genetic aberrant transcriptomic performances may contribute to inbreeding depression in P. yessoensis , deteriorating the stress tolerance and survival phenotype in self-bred progeny. (springer.com)
  • Myostatin expression was significantly decreased in quadriceps from mdx mice treated with rhEPO (rhEPO=0.60±0.11, control=1.07±0.11). (bvsalud.org)
  • The effects of myostatin/activin blocking using soluble activin receptor-Fc (sActRIIB-Fc) administration and wheel running were tested alone or in combination for 7 weeks in dystrophic mdx mice. (unboundmedicine.com)
  • Previous work suggested that increased expression of the dystrophin-related protein utrophin in the mdx mouse can reduce the dystrophic pathophysiology. (ox.ac.uk)
  • Although the increase in MLP and utrophin in the mdx mouse heart was able to compensate for the loss of dystrophin, accumulation of desmin, syncoilin and dystrophin were unable to compensate for the loss of MLP, resulting in heart failure. (ox.ac.uk)
  • Dystrophic mice and dogs had significantly higher cross-linked collagen than controls, especially in the diaphragm. (elsevier.com)
  • PURPOSE: To investigate the development of a laparoscopy technique for local injection into the X-linked muscular dystrophy (mdx) diaphragm. (bvsalud.org)
  • In this work, (1)H magnetic resonance spectroscopy (MRS) was used to study the metabolic profile of quadriceps and diaphragm muscles from mdx and control mice. (ox.ac.uk)
  • Diaphragm rescue alone prevents heart dysfunction in dystrophic mice. (ox.ac.uk)
  • In the dKO mouse, there was no difference in heart function between treatment of the diaphragm plus the heart and treatment of the diaphragm alone. (ox.ac.uk)
  • Mdx mice, which have the same dystrophin mutation as human patients, are of limited use, as they do not develop early dilated cardiomyopathy as seen in patients. (nature.com)
  • For decades, the majority of DMD research has been conducted using the mdx model, which has a mutation in the dystrophin gene itself like DMD patients. (nature.com)
  • To overcome this limitation, a number of double knockout mouse models have been developed that combine the dystrophin mutation of the mdx mouse with additional mutations. (nature.com)
  • Genetic correction of splice site mutation in purified and enriched myoblasts isolated from mdx5cv mice. (nih.gov)
  • The mdx5cv mouse is a model of DMD in which a point mutation in exon 10 of the dystrophin gene creates an artificial splice site. (nih.gov)
  • Using primary myoblasts from mdx5cv mice, single-stranded DNA oligonucleotides were designed to correct this DNA mutation. (nih.gov)
  • We also report that the frequency of genetic repair of the mdx mutation can be enhanced if RNAi is used to suppress expression of the recombinase inhibitor protein Msh2 in cultures containing myoblasts but not in those heavily enriched in myoblasts. (nih.gov)
  • A ) Schematic of the targeted exon of mouse Dmd and sequence from wild-type (upper) and mdx mice (lower).The mdx point mutation (C to T) is marked in red, and the premature stop codon is underlined. (nih.gov)
  • The treatment of DMD depends on gene mutation and molecular mechanism study of the disease, which requires reliable disease models such as mdx mouse model. (bvsalud.org)
  • We hypothesise that blockade of mast cell degranulation would reduce the extent of myofibre necrosis in the mdx mouse. (deepdyve.com)
  • Daily cromolyn injections were performed on young and exercised adult mdx mice and histological analysis confirmed that mast cell degranulation contributes to myofibre necrosis. (deepdyve.com)
  • An in vivo and in vitro H-magnetic resonance spectroscopy study of mdx mouse brain: abnormal development or neural necrosis? (ox.ac.uk)
  • Control mice were compared to mdx using a combination of in vivo and in vitro 1H-MRS methods to determine whether neural necrosis or developmental abnormalities occur in dystrophic brain. (ox.ac.uk)
  • NAA levels were normal in mdx brain compared to controls suggesting minor, if any, neuronal necrosis in dystrophic brain. (ox.ac.uk)
  • Abnormal cardiac function was accompanied by necrosis and lower citrate synthase activity in the mdx mouse heart, suggesting decreased mitochondrial content. (ox.ac.uk)
  • Subsequently, hDPSCs and hAFSCs, preliminarily demethylated and pre-differentiated toward a myogenic lineage for 2 weeks, were injected into the dystrophic gastrocnemius muscles of mdx/SCID mice. (pitt.edu)
  • Cy3) correct the dystrophin gene in cultures containing an enriched population of myoblasts isolated from dystrophic mice. (nih.gov)
  • Voluntary exercise in dystrophic mice enhances the markers of oxidative capacity and autophagy to or above the levels of healthy mice. (lu.se)
  • This novel mechanism of treating respiratory muscles to prevent cardiomyopathy in dystrophic mice warrants further investigation for its implications on the need to directly treat the heart in DMD. (ox.ac.uk)
  • Using dystrophin deficient mdx mouse and utrophin haplodeficient mdx/utrn +/- mouse models, we demonstrate the contribution of Dp427 (full-length dystrophin) and utrophin to testis and epididymis development, as well as spermatogenesis. (elsevier.com)
  • In this study, serum miRNA profiling reveals a distinct extracellular miRNA signature in dystrophin-deficient mdx mice, which shows profound dose-responsive restoration following dystrophin rescue. (ox.ac.uk)
  • Consequently, we characterized left-ventricular (LV) morphology and function in vivo using high-resolution cine-magnetic resonance imaging (MRI) in 2- to 3-month old dystrophin-deficient (mdx) and MLP-null mice, and their respective controls. (ox.ac.uk)
  • Here, we tested the hypothesis that SERCA2a over-expression may mitigate electrocardiography (ECG) abnormalities in old female mdx mice, a murine model of DMD cardiomyopathy. (biomedsearch.com)
  • The "long and the short of it" is that in a comparison to existing mouse models, the mdx 4cv /mTR G2 model with "humanized" somewhat shortened telomeres currently most closely approximates human DMD and will advance tests of therapeutic strategies. (nature.com)
  • We suggest that the inbred mice model presents an opportunity for researchers to investigate the genetic, and gene-environmental influence on the development of digit ratios. (jove.com)
  • Dystrophin deficiency in DMD/BMD patients and in the mdx mouse model appears to impair intracellular calcium homeostasis and to disrupt multiple protein-protein interactions that normally promote information transfer and signal integration from the extracellular environment to the nucleus within regulated microdomains. (elsevier.com)
  • Last month, Centre for Innovation Scientist Dr. Donald Branch along with the University of Toronto's Dr. Anton Neschadim published a new model " Mouse models for immune-mediated platelet destruction or immune thrombocytopenia" in Current Protocols. (blood.ca)
  • The mdx/utrn -/- mouse, lacking in both dystrophin and its autosomal homologue utrophin, is commonly used to model the clinical symptoms of DMD. (elsevier.com)
  • The mdx mouse is the classical animal model for DMD, showing similar molecular and protein defects. (ox.ac.uk)
  • The most active bi-specific CPP-PMOs demonstrated comparable exon skipping levels for both pre-mRNA targets when compared to individual CPP-PMO conjugates both in cell culture and in vivo in the mdx mouse model. (ox.ac.uk)
  • Here, we have determined whether the 8 month old mdx mouse, an animal model of muscular dystrophy, also has abnormal cardiac function and energetics. (ox.ac.uk)
  • 5. Ayers MM, Anderson R McD - Onion bulb neuropathy in the trembler mouse: a model for hypertrophic interstitial neuropathy (Dejerine-Sottas) in man. (scielo.br)
  • Pip5 transduction peptides direct high efficiency oligonucleotide-mediated dystrophin exon skipping in heart and phenotypic correction in mdx mice. (surrey.ac.uk)
  • Antisense oligonucleotide (AO)-mediated exon skipping has been shown to restore functional dystrophin in mdx mice and DMD patients treated intramuscularly in two recent phase 1 clinical trials. (surrey.ac.uk)
  • Immunofluorescence staining and western blot confirmed SERCA2a over-expression in the mdx heart. (biomedsearch.com)
  • To examine how inbreeding affects gene expression, we compared the transcriptome of two experimentally selfing families with inbreeding coefficient f reached 0.5 as well as one natural population ( f ≈ 0) of P. yessoensis . (springer.com)
  • 1989. Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice. (jax.org)
  • The donor splice site in the DMD gene in mdx5cv mice causes a frameshift which results in a translation of truncated dystrophin protein (10 of the 79 exons). (nih.gov)
  • Development of protein conjugate vaccines and testing in mice helped improve the meningitis Hib ( Haemophilus influenzae type b) vaccination for young children. (blood.ca)
  • Here, we have investigated the molecular architecture of dystrophin-like protein complexes in kidneys from normal and dystrophin-deficient mice. (ox.ac.uk)
  • We conclude that the absence of dystrophin in adult mdx mouse heart, as in the heart of human patient, is associated with right ventricular dilatation, left ventricular diastolic dysfunction and abnormal energy metabolism. (ox.ac.uk)
  • Mice homozygous for the mutant autosomal recessive gene "scid" which is located on the centromeric end of chromosome 16. (uchicago.edu)
  • SCID mice are useful as animal models since they are receptive to implantation of a human immune system producing SCID-human (SCID-hu) hematochimeric mice. (uchicago.edu)
  • Below are the most recent publications written about "Mice, SCID" by people in Profiles. (uchicago.edu)
  • Double knockout mouse models exhibit exacerbated disease phenotypes, but to variable degrees. (nature.com)
  • Six-month-old mdx mice showed right ventricular (RV) dilation and reduced RV ejection fraction (EF) in comparison to wild-type mice. (ox.ac.uk)
  • In vivo cardiac MRI revealed 33% and 104% larger right ventricular end-diastolic and end-systolic volumes, respectively, and 17% lower right ventricular ejection fractions in mdx mice compared with controls. (ox.ac.uk)
  • Abnormal cardiac morphology, function and energy metabolism in the dystrophic mdx mouse: an MRI and MRS study. (ox.ac.uk)
  • Mice, Jimpy" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
  • In line with this finding, treatment with the histone deacetylase inhibitor givinostat caused a significant increase in DMD transcript expression in mdx mice. (nih.gov)
  • A conundrum has been that although mdx mice lack dystrophin expression and exhibit chronic degeneration and regeneration of their myofibers, they do not manifest a number of symptoms of DMD. (nature.com)
  • 1995. Defective lymphoid development in mice lacking expression of the common cytokine receptor gamma chain. (jax.org)
  • Was found microscopically an increased number of lymph nodes and decreased in red pulp region by mdx, beyond a larger VEGF-C (vascular endothelial growth factor C) expression stimulates lymphangiogenesis in red pulp region from spleen. (bvsalud.org)
  • This article reviews the available information on expression of signaling-related molecules in DMD and mdx. (bvsalud.org)
  • Remarkable, through weighted gene co-expression network analysis (WGCNA), five common DEGs were found enriched in the most significant inbreeding related functional module M14 (FDR = 1.64E-156), including SREBP1 , G3BP2 , SBK1 , KIAA1161 , and AATs-Glupro . (springer.com)
  • Dystrophin- and MLP-deficient mouse hearts: marked differences in morphology and function, but similar accumulation of cytoskeletal proteins. (ox.ac.uk)
  • 2. Aguayo AJ, Attiwell M, et al - Abnormal myelination in transplanted mouse Schwann cells. (scielo.br)
  • On cryosections of dy/dy (f) mice, single necrotic fibers showed EBD staining by fluorescence microscopy. (nih.gov)
  • Nobel-winning scientific achievements such as the discovery of vitamin K, the development of the polio vaccine, the invention of monoclonal antibody technology now used for cancer treatment, and the unravelling of how neurons talk with each other in the brain all would not have occurred without mice. (blood.ca)
  • Analysis of gephyrin -/-, agrin -/-, and mdx mouse hippocampal neurons in culture indicated that synaptic clustering of dystroglycan occurs independently of gephyrin, agrin, and dystrophin. (wustl.edu)
  • Mdx mouse hearts contained significantly more fibrotic tissue than age-matched wild-type mouse hearts. (ox.ac.uk)
  • WT MDSCs implanted into the injured gastrocnemius of aged mdx mice significantly improved myofiber repair and reduced fat deposition and, to a lesser extent, fibrosis. (unboundmedicine.com)
  • or hemizygous for males) for the X-linked Dmd mdx-4Cv and IL2rγ null mutations. (jax.org)
  • Levels of focal adhesion kinase (FAK) and extracellular regulated kinases (ERKs) differ among mdx and DMD. (bvsalud.org)
  • Surprisingly, these mice, which have defects in the laminin alpha2-chain, an extracellular ligand of the DGC, showed little Evans blue accumulation in their skeletal muscles. (nih.gov)
  • Microarray analysis of mdx mice expressing high levels of utrophin: therapeutic implications for dystrophin deficiency. (ox.ac.uk)
  • CDCs were isolated and expanded from atrial biopsies from wild-type mice aged 1.5, 6, 18, and 24 months and from mdx mice aged 6 and 18 months. (ox.ac.uk)
  • Cardiac function was measured in mdx mice and age-matched wild-type mice using high-resolution cine magnetic resonance imaging. (ox.ac.uk)
  • Older mdx mice displayed significant RV and left ventricular dilation and decreased EF in both ventricles, compared with age-matched wild-type mice. (ox.ac.uk)
  • However, CDCs isolated from mice aged 6 and 18 months had the same number and regenerative potential from mdx mice and age-matched wild-type mice. (ox.ac.uk)
  • Amongst several animal species affected by neuromuscular disorders the house mouse is of particular interest because of its small size, short pregnancy and low costs of maintanence. (scielo.br)
  • Therefore, the aim of this study was to evaluate possible morphological changes from spleens in these mice and to compare with normal mice (Mus musculus) in contribution to DMD understanding and its consequences on immune system by affected individuals. (bvsalud.org)