| Full text | |
| Author(s): |
Engracia de Moraes, Julieta Rodini
;
Mouri Malvestio, Lygia Maria
;
Martins, Isabela Mancini
;
Erdmann Mosko, Patricia Regina
;
Engracia Filho, Jair Rodini
;
de Moraes, Flavio Ruas
Total Authors: 6
|
| Document type: | Journal article |
| Source: | Ciência Rural; v. 47, n. 10 2017. |
| Web of Science Citations: | 0 |
| Abstract | |
ABSTRACT: Golden Retriever muscular dystrophy (GRMD) is the most representative model for studying Duchenne muscular dystrophy (DMD) in humans, owing its phenotypic expression. DMD is a recessive disorder linked to the X chromosome in which the loss of dystrophin induces progressive weakness and degeneration of the skeletal and cardiac muscles, which lead to replacement by connective and adipose tissues. Onset of clinical signs occurs between 2 and 5 years of age, and many patients die from heart or respiratory failure. The main studies concerning dystrophic Golden Retrievers (DGR) sought to elucidate the pathophysiology of the disease and its clinical implications to develop therapies and alternative treatments to improve the quality of life and increase longevity of DMD patients. This review presents an overview of relevant contributions of the DGR model for elucidating DMD in humans. (AU) | |
| FAPESP's process: | 13/25957-6 - Morphological evaluation of cardiomyopathy in Golden Retriever dogs affected by muscular dystrophy |
| Grantee: | Lygia Maria Mouri Malvestio |
| Support Opportunities: | Scholarships in Brazil - Post-Doctoral |