scholarly journals Electrical impedance myography for the in vivo and ex vivo assessment of muscular dystrophy (mdx ) mouse muscle

2014 ◽  
Vol 49 (6) ◽  
pp. 829-835 ◽  
Author(s):  
Jia Li ◽  
Tom R. Geisbush ◽  
Glenn D. Rosen ◽  
Jennifer Lachey ◽  
Aaron Mulivor ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Electrical Impedance ◽  
Ex Vivo ◽  
Mdx Mouse ◽  
Mouse Muscle

scholarly journals Ultrasonography validation for early alteration of diaphragm echodensity and function in the mdx mouse model of Duchenne muscular dystrophy

PLoS ONE ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. e0245397
Author(s):  
Antonietta Mele ◽  
Paola Mantuano ◽  
Adriano Fonzino ◽  
Francesco Rana ◽  
Roberta Francesca Capogrosso ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Ex Vivo ◽  
Eccentric Contraction ◽  
Invasive Technique ◽  
Mdx Mouse ◽  
Early Stages ◽  
Over Time

The mdx mouse model of Duchenne muscular dystrophy is characterized by functional and structural alterations of the diaphragm since early stages of pathology, closely resembling patients’ condition. In recent years, ultrasonography has been proposed as a useful longitudinal non-invasive technique to assess mdx diaphragm dysfunction and evaluate drug efficacy over time. To date, only a few preclinical studies have been conducted. Therefore, an independent validation of this method by different laboratories is needed to increase results reliability and reduce biases. Here, we performed diaphragm ultrasonography in 3- and 6-month-old mdx mice, the preferred age-window for pharmacology studies. The alteration of diaphragm function over time was measured as diaphragm ultrasound movement amplitude. At the same time points, a first-time assessment of diaphragm echodensity was performed, as an experimental index of progressive loss of contractile tissue. A parallel evaluation of other in vivo and ex vivo dystrophy-relevant readouts was carried out. Both 3- and 6-month-old mdx mice showed a significant decrease in diaphragm amplitude compared to wild type (wt) mice. This index was well-correlated either with in vivo running performance or ex vivo isometric tetanic force of isolated diaphragm. In addition, diaphragms from 6-month-old dystrophic mice were also highly susceptible to eccentric contraction ex vivo. Importantly, we disclosed an age-dependent increase in echodensity in mdx mice not observed in wt animals, which was independent from abdominal wall thickness. This was accompanied by a notable increase of pro-fibrotic TGF-β1 levels in the mdx diaphragm and of non-muscle tissue amount in diaphragm sections stained by hematoxylin & eosin. Our findings corroborate the usefulness of diaphragm ultrasonography in preclinical drug studies as a powerful tool to monitor mdx pathology progression since early stages.

scholarly journals Relationships between in vivo surface and ex vivo electrical impedance myography measurements in three different neuromuscular disorder mouse models

PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0259071
Author(s):  
Sarbesh R. Pandeya ◽  
Janice A. Nagy ◽  
Daniela Riveros ◽  
Carson Semple ◽  
Rebecca S. Taylor ◽  
...  
Keyword(s):  
Electrical Impedance ◽  
Ex Vivo ◽  
Neuromuscular Disorders ◽  
Neuromuscular Disorder ◽  
Mdx Mouse ◽  
Obese Mouse ◽  
Moderate Degree ◽  
Frequency Range ◽  
Low Frequencies

Electrical impedance myography (EIM) using surface techniques has shown promise as a means of diagnosing and tracking disorders affecting muscle and assessing treatment efficacy. However, the relationship between such surface-obtained impedance values and pure muscle impedance values has not been established. Here we studied three groups of diseased and wild-type (WT) animals, including a Duchenne muscular dystrophy model (the D2-mdx mouse), an amyotrophic lateral sclerosis (ALS) model (the SOD1 G93A mouse), and a model of fat-related atrophy (the db/db diabetic obese mouse), performing hind limb measurements using a standard surface array and ex vivo measurements on freshly excised gastrocnemius muscle. A total of 101 animals (23 D2-mdx, 43 ALS mice, 12 db/db mice, and corresponding 30 WT mice) were studied with EIM across a frequency range of 8 kHz to 1 MHz. For both D2-mdx and ALS models, moderate strength correlations (Spearman rho values generally ranging from 0.3–0.7, depending on the impedance parameter (i.e., resistance, reactance and phase) were obtained. In these groups of animals, there was an offset in frequency with impedance values obtained at higher surface frequencies correlating more strongly to impedance values obtained at lower ex vivo frequencies. For the db/db model, correlations were comparatively weaker and strongest at very high and very low frequencies. When combining impedance data from all three disease models together, moderate correlations persisted (with maximal Spearman rho values of 0.45). These data support that surface EIM data reflect ex vivo muscle tissue EIM values to a moderate degree across several different diseases, with the highest correlations occurring in the 10–200 kHz frequency range. Understanding these relationships will prove useful for future applications of the technique of EIM in the assessment of neuromuscular disorders.

scholarly journals Investigating the Potential for Sulforaphane to Attenuate Gastrointestinal Dysfunction in mdx Dystrophic Mice

Nutrients ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 4559
Author(s):  
Kristy Swiderski ◽  
Suzannah J. Read ◽  
Audrey S. Chan ◽  
Jin D. Chung ◽  
Jennifer Trieu ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Corn Oil ◽  
Therapeutic Potential ◽  
Ex Vivo ◽  
In Vivo Studies ◽  
Muscle Pathology ◽  
Mdx Mouse ◽  
Mdx Mice ◽  
Dystrophic Mice

Gastrointestinal (GI) dysfunction is an important, yet understudied condition associated with Duchenne muscular dystrophy (DMD), with patients reporting bloating, diarrhea, and general discomfort, contributing to a reduced quality of life. In the mdx mouse, the most commonly used mouse model of DMD, studies have confirmed GI dysfunction (reported as altered contractility and GI transit through the small and large intestine), associated with increased local and systemic inflammation. Sulforaphane (SFN) is a natural isothiocyanate with anti-inflammatory and anti-oxidative properties via its activation of Nrf2 signalling that has been shown to improve aspects of the skeletal muscle pathology in dystrophic mice. Whether SFN can similarly improve GI function in muscular dystrophy was unknown. Video imaging and spatiotemporal mapping to assess gastrointestinal contractions in isolated colon preparations from mdx and C57BL/10 mice revealed that SFN reduced contraction frequency when administered ex vivo, demonstrating its therapeutic potential to improve GI function in DMD. To confirm this in vivo, four-week-old male C57BL/10 and mdx mice received vehicle (2% DMSO/corn oil) or SFN (2 mg/kg in 2% DMSO/corn oil) via daily oral gavage five days/week for 4 weeks. SFN administration reduced fibrosis in the diaphragm of mdx mice but did not affect other pathological markers. Gene and protein analysis revealed no change in Nrf2 protein expression or activation of Nrf2 signalling after SFN administration and oral SFN supplementation did not improve GI function in mdx mice. Although ex vivo studies demonstrate SFN’s therapeutic potential for reducing colon contractions, in vivo studies should investigate higher doses and/or alternate routes of administration to confirm SFN’s potential to improve GI function in DMD.

scholarly journals Investigation of the effect of taurine supplementation on muscle taurine content in the mdx mouse model of Duchenne muscular dystrophy using chemically specific synchrotron imaging

The Analyst ◽  
2020 ◽  
Vol 145 (22) ◽  
pp. 7242-7251
Author(s):  
Jessica R. Terrill ◽  
Samuel M. Webb ◽  
Peter G. Arthur ◽  
Mark J. Hackett
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Muscle Tissue ◽  
Mdx Mouse ◽  
Taurine Supplementation ◽  
Mouse Muscle ◽  
Synchrotron Imaging

Sulfur K-edge XANES was used to quantify changes in the taurine content of mouse muscle tissue in a model of muscular dystrophy. The changes could be associated with markers of disease pathology that were revealed by classical H&E histology.

scholarly journals In vivo MRI Characterization of Progressive Cardiac Dysfunction in the mdx Mouse Model of Muscular Dystrophy

PLoS ONE ◽  
2012 ◽  
Vol 7 (1) ◽  
pp. e28569 ◽  
Author(s):  
Daniel J. Stuckey ◽  
Carolyn A. Carr ◽  
Patrizia Camelliti ◽  
Damian J. Tyler ◽  
Kay E. Davies ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Mouse Model ◽  
Cardiac Dysfunction ◽  
Mdx Mouse

Direct retroviral-mediated transfer of a dystrophin minigene into mdx mouse muscle in vivo

1993 ◽  
Vol 2 (6) ◽  
pp. 717-723 ◽  
Author(s):  
Matthew G. Dunckley ◽  
Dominic J. Wells ◽  
Frank S. Walsh ◽  
George Dickson
Keyword(s):  
Mdx Mouse ◽  
Mouse Muscle

scholarly journals Microtubules that form the stationary lattice of muscle fibers are dynamic and nucleated at Golgi elements

2013 ◽  
Vol 203 (2) ◽  
pp. 205-213 ◽  
Author(s):  
Sarah Oddoux ◽  
Kristien J. Zaal ◽  
Victoria Tate ◽  
Aster Kenea ◽  
Shuktika A. Nandkeolyar ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Muscle ◽  
Muscle Diseases ◽  
Superresolution Microscopy ◽  
Nuclear Membranes ◽  
Organizing Center ◽  
Static Images

Skeletal muscle microtubules (MTs) form a nonclassic grid-like network, which has so far been documented in static images only. We have now observed and analyzed dynamics of GFP constructs of MT and Golgi markers in single live fibers and in the whole mouse muscle in vivo. Using confocal, intravital, and superresolution microscopy, we find that muscle MTs are dynamic, growing at the typical speed of ∼9 µm/min, and forming small bundles that build a durable network. We also show that static Golgi elements, associated with the MT-organizing center proteins γ-tubulin and pericentrin, are major sites of muscle MT nucleation, in addition to the previously identified sites (i.e., nuclear membranes). These data give us a framework for understanding how muscle MTs organize and how they contribute to the pathology of muscle diseases such as Duchenne muscular dystrophy.

scholarly journals In vivo cerebellar circuit function is disrupted in an mdx mouse model of Duchenne muscular dystrophy

2019 ◽  
Vol 13 (2) ◽  
pp. dmm040840 ◽  
Author(s):  
Trace L. Stay ◽  
Lauren N. Miterko ◽  
Marife Arancillo ◽  
Tao Lin ◽  
Roy V. Sillitoe
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Mdx Mouse ◽  
Circuit Function

scholarly journals Lmo7 is dispensable for skeletal muscle and cardiac function

2015 ◽  
Vol 309 (7) ◽  
pp. C470-C479 ◽  
Author(s):  
Dieu Hung Lao ◽  
Mary C. Esparza ◽  
Shannon N. Bremner ◽  
Indroneal Banerjee ◽  
Jianlin Zhang ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Cardiac Function ◽  
Cardiac Muscle ◽  
Mapk Pathway ◽  
Mdx Mouse ◽  
Mdx Mice ◽  
Mouse Line

Emery-Dreifuss muscular dystrophy (EDMD) is a degenerative disease primarily affecting skeletal muscles in early childhood as well as cardiac muscle at later stages. EDMD is caused by a number of mutations in genes encoding proteins associated with the nuclear envelope (e.g., Emerin, Lamin A/C, and Nesprin). Recently, a novel protein, Lim-domain only 7 ( lmo7) has been reported to play a role in the molecular pathogenesis of EDMD. Prior in vitro and in vivo studies suggested the intriguing possibility that Lmo7 plays a role in skeletal or cardiac muscle pathophysiology. To further understand the in vivo role of Lmo7 in striated muscles, we generated a novel Lmo7-null ( lmo7−/−) mouse line. Using this mouse line, we examined skeletal and cardiac muscle physiology, as well as the role of Lmo7 in a model of muscular dystrophy and regeneration using the dystrophin-deficient mdx mouse model. Our results demonstrated that lmo7−/− mice had no abnormalities in skeletal muscle morphology, physiological function, or regeneration. Cardiac function was also unaffected. Moreover, we found that ablation of lmo7 in mdx mice had no effect on the observed myopathy and muscular regeneration exhibited by mdx mice. Molecular analyses also showed no changes in dystrophin complex factors, MAPK pathway components, and Emerin levels in lmo7 knockout mice. Taken together, we conclude that Lmo7 is dispensable for skeletal muscle and cardiac physiology and pathophysiology.

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