Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle degeneration caused by the absence of dystrophin, a protein connecting the muscle fiber cytoskeleton to the extracellular matrix through the cell membrane. Symptoms which appear in boys by the age of 2 years old include a progressive decline in muscle strength and loss of muscle mass starting in the legs and pelvis extending to the arms and neck.
Several animal models are available but the most widely used in DMD research is the mdx mouse model. The mdx mouse model carries a nonsense point mutation in the exon 23 resulting in the premature translation termination of dystrophin expression. PsychoGenics has characterized the mdx mice in the presence and absence of a motor challenge exposure to fatigue the mice. Some of the data are presented on this page.
PsychoGenics maintains a colony of mdx mice to provide support preclinical studies.
PsychoGenics is also currently assessing the mdx4cv model [Chapman et al., PNAS 1989]. The mdx4cv has been generated using ENU mutagenesis resulting in point mutations in the dystrophin gene. The mdx4cv also carries a non sense mutation with a low background of reverent fibers like the mdx mice [Im et al, Human Molecular Genetics, 1996].
To view mdx mice phenotypic data, click on any of the experimental tests below:
Forced Treadmill Exercise
Running wheel activity in home cage