Histologic evaluation of the smooth muscle of Golden Retriever dogs affected by progressive muscular dystrophy (GRMD)

Abstract

Duchenne muscular dystrophy (DMD) is one of the most common inherited myopathies, recessive, and is linked to chromosome X. It is a degenerative myopathy characterized by the absence or dysfunction of dystrophin, a muscle membrane protein. Dogs Golden Retriever muscular dystrophy (GRMD) represents the best animal model available for future therapeutic trials aimed at the treatment of DMD in humans, due to the extensive homology between both pathogenicities. This project proposes the description of the histomorphological aspects of the smooth muscle of tubular organs of the gastrointestinal tract as well as bladder, ureters, and blood vessels of dogs GRMDs since the action of muscular dystrophy in striated skeletal muscle has been extensively studied, while action on the smooth muscle of this disease remains poorly understood. The results may be relevant to a better understanding of action and disease progression in this muscle group, allowing the development of possible new approaches to treat the same, thus enabling a better quality of life of individuals affected by the disease.(AU)