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Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice

Mucopolysaccharidosis I (MPS I), known as Hurler syndrome in the severe form, is a lysosomal storage disease due to α-l-iduronidase (IDUA) deficiency. It results in fragmentation of elastin fibers in the aorta and heart valves via mechanisms that are unclear, but may result from the accumulation of...

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Detalhes bibliográficos
Main Authors: Ma, Xiucui, Tittiger, Mindy, Knutsen, Russell H., Kovacs, Attila, Schaller, Laura, Mecham, Robert P., Ponder, Katherine P.
Formato: Artigo
Idioma:Inglês
Publicado em: 2008
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3775334/
https://ncbi.nlm.nih.gov/pubmed/18479957
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2008.03.018
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