Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation

Mucopolysaccharidosis VII (MPS VII) is due to mutations within the gene encoding the lysosomal enzyme β-glucuronidase, and results in the accumulation of glycosaminoglycans. MPS VII causes aortic dilatation and elastin fragmentation, which is associated with upregulation of the elastases cathepsin S...

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Detalhes bibliográficos
Main Authors: Baldo, Guilherme, Wu, Susan, Howe, Ruth A., Ramamoothy, Meera, Knutsen, Russell H., Fang, Jiali, Mecham, Robert P., Liu, Yuli, Wu, Xiaobo, Atkinson, John P., Ponder, Katherine P.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3283036/
https://ncbi.nlm.nih.gov/pubmed/21944884
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2011.08.018
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