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Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans Ciência Rural
Moraes,Julieta Rodini Engrácia de; Malvestio,Lygia Maria Mouri; Martins,Isabela Mancini; Mosko,Patrícia Regina Erdmann; Engracia Filho,Jair Rodini; Moraes,Flávio Ruas de.
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...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Duchenne muscular dystrophy; Animal model; Dystrophic Golden Retriever.
Ano: 2017 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503
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