Duchenne muscular dystrophy - page 2 Scientific Publications

Latest publication 09/21/2020

A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd d

Duchenne muscular dystrophy (DMD) is a life-threatening neuromuscular disease caused by the lack of dystrophin, resulting in progressive muscle...

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    [title] => A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd d
    [paragraph] => A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd deletion exhibits progressive muscular dystrophy and early-onset cardiomyopathy
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Authors
TWY Wong, A Ahmed, G Yang et al 

 Lab
University of Toronto, Toronto, Canada
Journal
Disease Models & Mechanisms
Abstract
Duchenne muscular dystrophy (DMD) is a life-threatening neuromuscular disease caused by the lack of dystrophin, resulting in progressive muscle wasting and locomotor dysfunctions. By adulthood, almost all patients also develop cardiomyopathy, which is the primary cause of death in DMD. Although there has been extensive effort in creating animal models to study treatment strategies for DMD, most fail to recapitulate the complete skeletal and cardiac disease manifestations that are presented in affected patients. Here, we generated a mouse model mirroring a patient deletion mutation of exons 52-54 (Dmd delta52-54). The Dmd delta52-54 mutation led to the absence of dystrophin, resulting in progressive muscle deterioration with weakened muscle strength. Moreover, Dmd delta52-54 mice present with early-onset hypertrophic cardiomyopathy, which is absent in current pre-clinical dystrophin-deficient mouse models. Therefore, Dmd delta52-54 presents itself as an excellent preclinical model to evaluate the impact on skeletal and cardiac muscles for both mutation-dependent and -independent approaches.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)

Keywords/Topics
Duchenne muscular dystrophy; Cardiac function
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An easy way to objectively quantify the muscular strength of mice and rats, and to assess the effect of drugs, toxins, muscular (i.e. myopathy) and neurodegenerative diseases on muscular degeneration. It is widely used in conjunction with the ROTAROD motor coordination test: a normally coordinated rodent will show a decreased latency to fall off the rotating rod if its muscular strength is low. The Grip Strength Test is a must for your research on activity, motor control & coordination, and is particularly well suited for studies on Parkinson's & Huntington's disease.
New features GS4 - 2023: Color display with permanent backlight screen for easier reading, reset by footswitch, Improved battery time, Larger data memory of 500 values, Animal counter, USB port (charging/data transfer)

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