1. Relevant aspects of golden retriever muscular dystrophy for the study of duchenne muscular dystrophy in humans
- Author
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De Moraes, Julieta Rodini Engrácia [UNESP], Malvestio, Lygia Maria Mouri, Martins, Isabela Mancini [UNESP], Mosko, Patrícia Regina Erdmann, Filho, Jair Rodini Engracia, De Moraes, Flávio Ruas [UNESP], Universidade Estadual Paulista (Unesp), Faculdade Meridional (IMED), and Pontifícia Universidade Católica do Paraná (PUC-PR)
- Subjects
Duchenne muscular dystrophy ,Animal model ,Dystrophic golden retriever - Abstract
Made available in DSpace on 2018-12-11T16:49:17Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-01-01. Added 1 bitstream(s) on 2019-10-09T18:29:25Z : No. of bitstreams: 1 S0103-84782017001000503.pdf: 540800 bytes, checksum: dad4ecf03c54561ddec0cc0ef0b89863 (MD5) Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) 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. Faculdade de Ciências Agrárias e Veterinárias (FCAV) Universidade Estadual Paulista (UNESP) Escola de Medicina Veterinária Faculdade Meridional (IMED) Pontifícia Universidade Católica do Paraná (PUC-PR) Faculdade de Ciências Agrárias e Veterinárias (FCAV) Universidade Estadual Paulista (UNESP) FAPESP: 13/25957-6
- Published
- 2017