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Provedor de dados:  Ciência Rural
País:  Brazil
Título:  Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
Autores:  Moraes,Julieta Rodini Engrácia de
Malvestio,Lygia Maria Mouri
Martins,Isabela Mancini
Mosko,Patrícia Regina Erdmann
Engracia Filho,Jair Rodini
Moraes,Flávio Ruas de
Data:  2017-01-01
Ano:  2017
Palavras-chave:  Duchenne muscular dystrophy
Animal model
Dystrophic Golden Retriever
Resumo:  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.
Tipo:  Info:eu-repo/semantics/article
Idioma:  Inglês
Identificador:  http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503
Editor:  Universidade Federal de Santa Maria
Relação:  10.1590/0103-8478cr20160470
Formato:  text/html
Fonte:  Ciência Rural v.47 n.10 2017
Direitos:  info:eu-repo/semantics/openAccess
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