Pulmonary Abnormalities in Animal Models Due to Niemann-Pick Type C1 (NPC1) or C2 (NPC2) Disease

Niemann-Pick C (NPC) disease is due to loss of NPC1 or NPC2 protein function that is required for unesterified cholesterol transport from the endosomal/lysosomal compartment. Though lung involvement is a recognized characteristic of Niemann-Pick type C disease, the pathological features are not well...

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Main Authors: Roszell, Blair R., Tao, Jian-Qin, Yu, Kevin J., Gao, Ling, Huang, Shaohui, Ning, Yue, Feinstein, Sheldon I., Vite, Charles H., Bates, Sandra R.
Format: Artigo
Sprog:Inglês
Udgivet: Public Library of Science 2013
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3699545/
https://ncbi.nlm.nih.gov/pubmed/23843985
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0067084
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