* Interest of the canine models in hereditary myopathies 

* Characterization of hereditary  myopathies

* Research of therapeutic processes

* Development of tools of functional evaluations


First specie domesticated by human, the dog has undergone a huge pressure of selection during the centuries. The genetic crosses, performed by humans to create races responding to various requirements (hunting, guarding, people assistance, etc), has produced a diversity that no other mammalian species offers. Unfortunately, this selection based on morphological or behavioral attributes was accompanied by the selection of defective genes responsible for several genetic diseases among which are myopathies.

The canine myopathies are often quasi perfect replications of the human diseases. For example, the golden retriever muscular dystrophy (GRMD) is a homologous disease to the Duchenne muscular dystrophy. The GRMD dogs carry, like human patients, mutations in the dystrophin gene. From the clinical and the histological point of view, they display important locomotive affections, respiratory and cardiac failures, necrotic lesions and fibrosis similar to those observed in human. These strong phenotypic and genetics homologies between human and canine patients underline the relevance of the dog as study model.
In addition, there are significant advantages to involve canine models in the research on myopathies, and especially at a preclinical level. First, to be efficient, a treatment against myopathy needs to reach all dystrophic muscles. The analysis of the bio-distribution of the administrated medication is thus an unavoidable preclinical step. On this aspect, dogs, with a size equivalent to a sick child, constitute an appropriate model. Next, they allow a reliable and quantified evaluation of motor functions, contrary to mice which badly reproduce symptomatic tables of human myopathies. Finally, it is easy to follow the global physiology in dogs. Indeed, clinical examinations and specialized complementary examinations (echo-cardiographic exams for example) are available in this specie.
However, the dog is a recent model; the research tools available in this specie are relatively scarce. Moreover, it is an extremely delicate model requiring a specialized structure and permanent veterinary cares.