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AutorBarthélémy, Inês
AutorMariz, Fernanda Pinto
AutorYada, Erica
AutorDesquilbet, Loïc
AutorSavino, Wilson
AutorBarbosa, Suse Dayse Silva
AutorFaussat, Anne-Marie
AutorMouly, Vincent
AutorVoit, Thomas
AutorBlot, Stéphane
AutorButler-Browne, Gillian
Data de acesso2015-06-08T14:01:46Z
Data de disponibilização2015-06-08T14:01:46Z
Data do publicação2014
CitaçãoBARTHÉLÉMY, Inès et al. Predictive markers of clinical outcome in the GRMD dog model of Duchenne muscular dystrophy. Disease Models & Mechanisms, v.7, n.11, p.1253-1261, 2014.pt_BR
ISSN1754-8403pt_BR
URIhttps://www.arca.fiocruz.br/handle/icict/10676
Idiomaengpt_BR
EditorThe Company of Biologists Ltdpt_BR
Direito Autoralopen access
Palavras-chaveLinfócitospt_BR
TítuloPredictive markers of clinical outcome in the GRMD dog model of Duchenne muscular dystrophypt_BR
Tipo do documentoArticle
DOI10.1242/dmm.016014pt_BR
Resumo em InglêsIn the translational process of developing innovative therapies for DMD (Duchenne muscular dystrophy), the last preclinical validation step is often carried out in the most relevant animal model of this human disease, namely the GRMD (Golden Retriever muscular dystrophy) dog. The disease in GRMD dogs mimics human DMD in many aspects, including the inter-individual heterogeneity. This last point can be seen as a drawback for an animal model but is inherently related to the disease in GRMD dogs closely resembling that of individuals with DMD. In order to improve the management of this inter-individual heterogeneity, we have screened a combination of biomarkers in sixty-one 2-month-old GRMD dogs at the onset of the disease and a posteriori we addressed their predictive value on the severity of the disease. Three non-invasive biomarkers obtained at early stages of the disease were found to be highly predictive for the loss of ambulation before 6 months of age. An elevation in the number of circulating CD4+CD49dhi T cells and a decreased stride frequency resulting in a reduced spontaneous speed were found to be strongly associated with the severe clinical form of the disease. These factors can be used as predictive tests to screen dogs to separate them into groups with slow or fast disease progression before their inclusion into a therapeutic preclinical trial, and therefore improve the reliability and translational value of the trials carried out on this invaluable large animal model. These same biomarkers have also been described to be predictive for the time to loss of ambulation in boys with DMD, strengthening the relevance of GRMD dogs as preclinical models of this devastating muscle disease.pt_BR
AfiliaçãoUniversité Paris-Est. Ecole Nationale Vétérinaire d’Alfort. UPR de Neurobiologie. Paris, France.pt_BR
AfiliaçãoUniversité Pierre et Marie Curie-Paris. Institut de Myologie. Paris, France/ Universidade Federal do Rio de Janeiro. Instituto de Pediatria. Rio de Janeiro, RJ, Brasil / Fundação Oswaldo Cruz. Instituto Oswaldo Cruz. Laboratório de Pesquisa sobre o Timo. Rio de Janeiro, RJ, Brasil.pt_BR
AfiliaçãoUniversité Pierre et Marie Curie-Paris . Institut de Myologie. Paris, France.pt_BR
AfiliaçãoUniversité Paris-Est. Ecole Nationale Vétérinaire d’Alfort. Unité D’Épidémiologie clinique et de Biostatistique. Paris, France / CNRS UMR 7179, MNHN, Brunoy 91800, France.pt_BR
AfiliaçãoFundação Oswaldo Cruz. Instituto Oswaldo Cruz. Laboratório de Pesquisa sobre o Timo. Rio de Janeiro, RJ, Brasil.pt_BR
AfiliaçãoInstituto Nacional de Câncer (INCA). Departamento de Pesquisa Clínica. Rio de Janeiro, RJ, Brasil.pt_BR
AfiliaçãoUniversité Pierre et Marie Curie-Paris . Institut de Myologie. Paris, France.pt_BR
AfiliaçãoUniversité Pierre et Marie Curie-Paris . Institut de Myologie. Paris, France.pt_BR
AfiliaçãoUniversité Paris-Est. Ecole Nationale Vétérinaire d’Alfort. UPR de Neurobiologie. Paris, France.pt_BR
AfiliaçãoUniversité Pierre et Marie Curie-Paris . Institut de Myologie. Paris, France.pt_BR
Palavras-chave em inglêsGRMDpt_BR
Palavras-chave em inglêsDMDpt_BR
Palavras-chave em inglêsDistrophinpt_BR
Palavras-chave em inglêsPredictive biomakerpt_BR
Palavras-chave em inglêsLymphocytept_BR
Palavras-chave em inglêsCD49dpt_BR
Palavras-chave em inglêsGait analysispt_BR
Palavras-chave em inglêsAccelerometrypt_BR
DeCSAcelerometriapt_BR
DeCSDystrophinpt_BR


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