Owing to advancements in genetic engineering, a wide variety of mouse models are available for the majority of MDs. Here, we summarize the characteristics of the most commonly used mouse models for a subset of highly studied MDs, collated into a table.

The Jackson Laboratory has a large collection of mouse models of muscular dystrophy. They are categorized by specific disease type to help you easily find the best strain for your research.

Here we characterized disease progression of three mutant mouse strains of muscular dystrophy: mdx mice, which lack dystrophin; mdx:utrn-/- mice, which also lack utrophin; and dy/dy mice, which are deficient in laminin alpha2.

One of the mouse models for laminin α2 chain-deficiency, the spontaneous dy/dy mutant, was described in 1955. It was the first reported mouse strain representing the pathological criteria that were characteristic for muscular dystrophy.

We further show that separate treatment with two antioxidant drugs (both approved for human use) prevents muscular dystrophy progression in the dy 2J /dy 2J mouse model of LAMA2-CMD.

In the dy W strain (B6.129S1 (Cg)- Lama2 tm1Eeng /J, 013786), knock-out of the laminin alpha 2 gene models merosin-deficient congenital muscular dystrophy. Homozygous dy W mice are passive, small, and emaciated, and demonstrate partial hindleg lameness and clasping.

These dy W mice may be useful for studying merosin-deficient congenital muscular dystrophy and other muscular dystrophies. Our preclinical efficacy testing services offer scientific expertise and an array of target-based and phenotype-based outcome measures, both in vivo and at endpoint, for flexible study designs and assay development in mouse ...

Nov 1, 2001 · Here we characterized disease progression of three mutant mouse strains of muscular dystrophy: mdx mice, which lack dystrophin; mdx:utrn−/− mice, which also lack utrophin; and dy/dy mice, which are deficient in laminin α2.

Duchenne's muscular dystrophy (DMD) is a lethal childhood disease caused by mutations of the dystrophin gene, the protein product of which, dystrophin, has a vital role in maintaining muscle structure and function. Homologues of DMD have been identified in several animals including dogs, cats, mice, fish and invertebrates.

Jul 1, 2002 · We investigated the dy mouse model, which has congenital muscular dystrophy due to a defect in laminin α2, for evidence of cochlear dysfunction. Auditory brainstem response (ABR) audiometry to pure tones was used to evaluate 3-month-old homozygous dy/dy and age-matched C57 control mice.