Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans

Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans

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.

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Main Authors: Moraes,Julieta Rodini Engrácia de, Malvestio,Lygia Maria Mouri, Martins,Isabela Mancini, Mosko,Patrícia Regina Erdmann, Engracia Filho,Jair Rodini, Moraes,Flávio Ruas de
Format: Digital revista
Language:English
Published: Universidade Federal de Santa Maria 2017
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503
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spelling oai:scielo:S0103-847820170010005032018-01-04Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humansMoraes,Julieta Rodini Engrácia deMalvestio,Lygia Maria MouriMartins,Isabela ManciniMosko,Patrícia Regina ErdmannEngracia Filho,Jair RodiniMoraes,Flávio Ruas de Duchenne muscular dystrophy animal model dystrophic Golden Retriever 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.info:eu-repo/semantics/openAccessUniversidade Federal de Santa MariaCiência Rural v.47 n.10 20172017-01-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503en10.1590/0103-8478cr20160470
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country Brasil
countrycode BR
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region America del Sur
libraryname SciELO
language English
format Digital
author Moraes,Julieta Rodini Engrácia de
Malvestio,Lygia Maria Mouri
Martins,Isabela Mancini
Mosko,Patrícia Regina Erdmann
Engracia Filho,Jair Rodini
Moraes,Flávio Ruas de
spellingShingle Moraes,Julieta Rodini Engrácia de
Malvestio,Lygia Maria Mouri
Martins,Isabela Mancini
Mosko,Patrícia Regina Erdmann
Engracia Filho,Jair Rodini
Moraes,Flávio Ruas de
Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
author_facet Moraes,Julieta Rodini Engrácia de
Malvestio,Lygia Maria Mouri
Martins,Isabela Mancini
Mosko,Patrícia Regina Erdmann
Engracia Filho,Jair Rodini
Moraes,Flávio Ruas de
author_sort Moraes,Julieta Rodini Engrácia de
title Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
title_short Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
title_full Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
title_fullStr Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
title_full_unstemmed Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans
title_sort relevant aspects of golden retriever muscular dystrophy for the study of duchenne muscular dystrophy in humans
description 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.
publisher Universidade Federal de Santa Maria
publishDate 2017
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0103-84782017001000503
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AT martinsisabelamancini relevantaspectsofgoldenretrievermusculardystrophyforthestudyofduchennemusculardystrophyinhumans
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