Corticortophin releasing factor 2 receptor agonist treatment significantly slows disease progression in mdx mice.

ABSTRACT:

Background

Duchenne muscular dystrophy results from mutation of the dystrophin gene, causing skeletal and cardiac muscle loss of function. The mdx mouse model of Duchenne muscular dystrophy is widely utilized to evaluate the potential of therapeutic regimens to modulate the loss of skeletal muscle function associated with dystrophin mutation. Importantly, progressive loss of diaphragm function is the most consistent striated muscle effect observed in the mdx mouse model, which is the same as in patients suffering from Duchenne muscular dystrophy.

Methods

Using the mdx mouse model, we have evaluated the effect that corticotrophin releasing factor 2 receptor (CRF2R) agonist treatment has on diaphragm function, morphology and gene expression.

Results

We have observed tha

SUBMITTER: Hinkle RT 

PROVIDER: S-EPMC1936998 | biostudies-literature | 2007 Jul

REPOSITORIES: biostudies-literature