Mice inbred mdx. Medical search. Frequent questions

The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR DYSTROPHY. (uchicago.edu)
Of Mice and Measures": A Project to Improve How We Advance Duchenne Muscular Dystrophy Therapies to the Clinic. (uchicago.edu)
Skeletal muscle fibrosis in the mdx/utrn+/- mouse validates its suitability as a murine model of Duchenne muscular dystrophy. (uchicago.edu)
In this paper we constructed a simple mathematical model to investigate the role of the immune response in muscle degeneration and subsequent regeneration in the mdx mouse model of Duchenne muscular dystrophy. (hindawi.com)
The X chromosome-linked mutation resulted in mice ( mdx mice) with high serum levels of muscle enzymes and with histological lesions comparable to those seen in human muscular dystrophy. (hindawi.com)
Materials & methods: We have isolated wild-type mesoangioblasts from aorta and tested their effectiveness in alleviating severe muscle disease in the dystrophin/utrophin knockout (mdx/utrn -l- ) mouse model for Duchenne muscular dystrophy. (illinois.edu)
An alternative strategy circumventing many problems associated with somatic gene therapies for Duchenne muscular dystrophy has arisen from the demonstration that utrophin can functionally substitute for dystrophin and its over-expression in muscles of dystrophin-null transgenic mice completely prevents the phenotype arising from dystrophin deficiency. (ox.ac.uk)
Here we show that co-administration of phosphorodiamidate morpholino oligomers (PMOs) with glucose enhances exon-skipping activity in Duchenne muscular dystrophy (DMD) mdx mice. (ox.ac.uk)
To facilitate gene and cell therapy experiments, we created severely immune-deficient mouse models of Duchenne muscular dystrophy (DMD), limb girdle muscular dystrophy 2B (LGMD2B), and limb girdle muscular dystrophy 2D (LGMD2D) by crossing mdx 4Cv , Bl/AJ, and Sgca-null mice with NRG immune-deficient mice. (plos.org)
Using this technique the mdx mouse, a model of Duchenne muscular dystrophy (DMD) was examined. (ox.ac.uk)
Here we show that increasing the expression of intramuscular heat shock protein 72 (Hsp72) preserves muscle strength and ameliorates the dystrophic pathology in two mouse models of muscular dystrophy. (ox.ac.uk)
Assessment of Behavioral Characteristics With Procedures of Minimal Human Interference in the mdx Mouse Model for Duchenne Muscular Dystrophy. (sylics.com)

Expression of a myelin basic protein gene in transgenic shiverer mice: correction of the dysmyelinating phenotype. (harvard.edu)
By contrast, the phenotype of the BlAJ/NRG mice was milder in each case. (plos.org)
To determine their therapeutic value, dystrophic mdx mice were subject to forced exercise to model the DMD cardiac phenotype. (ox.ac.uk)

One of the first INBRED MOUSE STRAINS to be sequenced. (uchicago.edu)

In marked contrast, a dystrophin protein lacking the cysteine-rich domain, which is unable to prevent dystrophy in the mdx mouse, is able to ameliorate these abnormalities in utrophin/dystrophin-deficient mice. (ox.ac.uk)

This strain is commonly used as genetic background for transgenic mouse models. (uchicago.edu)
Protein structure predictions, patient mutations, in vitro binding studies and transgenic and knockout mice suggest that dystrophin plays a mechanical role in skeletal muscle, linking the subsarcolemmal cytoskeleton with the extracellular matrix through its direct interaction with the dystrophin-associated protein complex (DAPC). (ox.ac.uk)

A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. (uchicago.edu)
identified a spontaneous mutation in C57BL/10ScSn inbred mice that exhibited a disease state similar to human DMD [ 4 ]. (hindawi.com)
Mice, Inbred C57BL" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (uchicago.edu)
Below are the most recent publications written about "Mice, Inbred C57BL" by people in Profiles. (uchicago.edu)

A Protocol for Simultaneous In Vivo Imaging of Cardiac and Neuroinflammation in Dystrophin-Deficient MDX Mice Using [18F]FEPPA PET. (uchicago.edu)
Hexose enhances oligonucleotide delivery and exon skipping in dystrophin-deficient mdx mice. (ox.ac.uk)

Treatment with BGP-15 (a pharmacological inducer of Hsp72 currently in clinical trials for diabetes) improved muscle architecture, strength and contractile function in severely affected diaphragm muscles in mdx dystrophic mice. (ox.ac.uk)
In dko mice, a phenocopy of DMD that results in severe spinal curvature (kyphosis), muscle weakness and premature death, BGP-15 decreased kyphosis, improved the dystrophic pathophysiology in limb and diaphragm muscles and extended lifespan. (ox.ac.uk)
We found that the sarcoplasmic/endoplasmic reticulum Ca(2+)-ATPase (SERCA, the main protein responsible for the removal of intracellular Ca(2+)) is dysfunctional in severely affected muscles of mdx and dko mice, and that Hsp72 interacts with SERCA to preserve its function under conditions of stress, ultimately contributing to the decreased muscle degeneration seen with Hsp72 upregulation. (ox.ac.uk)

To directly assess the role of nNOS in mdz muscle, we generated dystrophin-nNOS double knockout mice by mating nNOS single knockout mice with mdx mice. (johnshopkins.edu)
The double knockout mice are indistinguishable from the rndx mice in terms of dystrophic muscle morphology and sarcolemma membrane damage. (johnshopkins.edu)
To identify potential non-mechanical roles of dystrophin, we tested the ability of various truncated dystrophin transgenes to prevent any of the skeletal muscle abnormalities associated with the double knockout mouse deficient for both dystrophin and the dystrophin-related protein utrophin. (ox.ac.uk)

This mutation in the murine dystrophin gene caused an absence of dystrophin in skeletal muscle and this key defect validated the mdx mouse as a suitable model of the early onset of DMD human disease [ 5 , 6 ]. (hindawi.com)

In contrast, Noggin was found to be a potent and selective BMP inhibitor and was therefore tested in vivo in a DMD mouse model. (nih.gov)
During early disease onset, the mdx mouse has been validated as an animal model for DMD and use of this model has led to valuable but incomplete insights into the disease process. (hindawi.com)
Furthermore, the analysis of the model predicts that the immune system response oscillates throughout the life of the mice, and the damaged fibers are never completely cleared. (hindawi.com)
The histology and time course of the disease in mdx mouse model are very different from those in DMD patients: relatively normal life span and overall fitness compared to progressive physical impairment leading to death in DMD patients [ 7 ]. (hindawi.com)
Nonetheless, the mdx mouse model is regarded as the best animal model, especially of the early onset of DMD. (hindawi.com)
The article [ 13 ] is a comprehensive literature review of the immune-mediated molecular and signaling mechanisms that regulate the time course of the disease and the mdx mouse model. (hindawi.com)
Single CAR-T cell treatment controls disseminated ovarian cancer in a syngeneic mouse model. (uchicago.edu)
Structural bases for central nervous system malfunction in the quaking mouse: dysmyelination in a potential model of schizophrenia. (harvard.edu)
We performed centronucleation, Evans blue dye, hydroxyproline, and treadmill assays on the disease model mice versus NRG controls to evaluate muscle histology and function. (plos.org)
These data define the dystrophin restoration levels required to slow down or prevent disease progression and improve overall muscle function once a dystrophic environment has been established in the mdx mouse model. (ox.ac.uk)
Functional brain defects in a mouse model of a chromosomal t(1;11) translocation that disrupts DISC1 and confers increased risk of psychiatric illness. (sylics.com)
Hyperactivity, perseveration and increased responding during attentional rule acquisition in the Fragile X mouse model. (sylics.com)

Mice, Inbred mdx" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (uchicago.edu)

These novel immune-deficient mouse models of DMD, LGMD2B, and LGMD2D will be useful for long-term gene and cell therapy studies involving transfer of foreign genes and cells. (plos.org)

Treated mice also exhibited significantly reduced cardiac fibrosis and improved sarcolemmal integrity. (ox.ac.uk)

These results are in line with previous data and suggest that before the onset of myofiber necrosis, mitochondrial and metabolic abnormalities are present within the mdx mouse. (frontiersin.org)

Long-term wheel running compromises diaphragm function but improves cardiac and plantarflexor function in the mdx mouse. (uchicago.edu)
This work demonstrates that high levels of cardiac dystrophin restored by Pip peptide-AOs prevents further deterioration of cardiomyopathy and pathology following exercise in dystrophic DMD mice. (ox.ac.uk)

Lastly, inhibition of the strong autophagic program observed in adolescent mdx male mice via administration of the autophagy inhibitor leupeptin did not improve skeletal muscle pathology. (frontiersin.org)
These results indicate that the mdx pathology does not directly result from aberrant localization of nNOS. (johnshopkins.edu)

In addition, these mice provide unique insights into skeletal muscle fiber type composition. (ox.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. (ox.ac.uk)

Genetic Variation in CNS Myelination and Functional Brain Connectivity in Recombinant Inbred Mice. (sylics.com)

Proton magnetic resonance in myelin deficient brains of mutant mice. (harvard.edu)
Myelin deficient mice: expression of myelin basic protein and generation of mice with varying levels of myelin. (harvard.edu)
Collectively, we show that GF potentiates PMO activity by replenishing cellular energy stores under energy-deficient conditions in mdx mice. (ox.ac.uk)
Altered phase-relationship between peripheral oscillators and environmental time in Cry1 or Cry2 deficient mouse models for early and late chronotypes. (sylics.com)

In this study, we detected aberrant mitochondrial morphology, reduced cristae number, and large mitochondrial vacuoles from both male and female mdx mice prior to the onset of muscle damage. (frontiersin.org)
We further detected mild metabolic and mitochondrial impairments in female mdx carrier mice that were exacerbated with high-fat diet feeding. (frontiersin.org)

These studies demonstrated that the mdx 4Cv /NRG and Sgca/NRG mice showed significant deficits in muscle structure and function in all the assays and were similar to each other. (plos.org)
Epileptiform activity and cognitive deficits in SNAP-25(+/-) mice are normalized by antiepileptic drugs. (sylics.com)

Hence, nNOS absence in mdx and DMD tissue is likely a secondary effect to the loss of dystrophin and does not participate in the disease pathogenesis. (johnshopkins.edu)

Mice homozygous for the mutant autosomal recessive gene, quaking (qk), associated with disorder in myelin formation and manifested by axial tremors. (harvard.edu)
Size changes of protamine 1 mRNA provide a molecular marker to monitor spermatogenesis in wild-type and mutant mice. (harvard.edu)
The resulting mdx 4Cv /NRG, BlAJ/NRG, and Sgca/NRG mice demonstrated the presence of the appropriate mutant alleles at Dmd , Dysf , Sgca , Rag1 , and Il2 rγ by genotyping PCR. (plos.org)
Mice bearing mutant genes which are phenotypically expressed in the animals. (ucdenver.edu)

Pip5 transduction peptides direct high efficiency oligonucleotide-mediated dystrophin exon skipping in heart and phenotypic correction in mdx mice. (ox.ac.uk)

Effects of Depilatory Cream Formulation and Contact Time on Mouse Skin. (uchicago.edu)

Prevention of exercised induced cardiomyopathy following Pip-PMO treatment in dystrophic mdx mice. (ox.ac.uk)

Immunoglobulin G subclasses confer protection against Staphylococcus aureus bloodstream dissemination through distinct mechanisms in mouse models. (uchicago.edu)

In order to robustly evaluate functional improvement, we used in situ protocols in the mdx mouse to measure muscle strength and resistance to eccentric contraction-induced damage. (ox.ac.uk)

Below are the most recent publications written about "Mice, Inbred mdx" by people in Profiles. (uchicago.edu)

This graph shows the total number of publications written about "Mice, Inbred mdx" by people in this website by year, and whether "Mice, Inbred mdx" was a major or minor topic of these publications. (uchicago.edu)

Munc18-1 haploinsufficiency results in enhanced anxiety-like behavior as determined by heart rate responses in mice. (sylics.com)

How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse. (ox.ac.uk)

Loos M, Staal J, Smit AB, De Vries TJ, Spijker S. Enhanced alcohol self-administration and reinstatement in a highly impulsive, inattentive recombinant inbred mouse strain. (sylics.com)

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MUSCULAR DYSTROPHY12

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C57BL4

Dystrophin-deficient2

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Double knockout3

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Cardiac2

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