Pulmonary Function and Pathology in Hydroxypropyl-beta-cyclodextin-treated and Untreated Npc1(−/−) Mice

Lung dysfunction is an important part of the pathology of the neurodegenerative disorder, Niemann-Pick C1 (NPC1). We have studied the pulmonary disease in the Npc1(NIH/NIH) mouse model. On histology, we find large numbers of alveolar foamy macrophages but no alveolar proteinosis. Lung weight as perc...

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Detalhes bibliográficos
Main Authors: Muralidhar, Akshay, Borbon, Ivan A, Esharif, Dyadin M., Ke, Wangjing, Manacheril, Rinu, Daines, Michael, Erickson, Robert P
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3107736/
https://ncbi.nlm.nih.gov/pubmed/21459030
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2011.03.001
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