Pulmonary Abnormalities in Animal Models Due to Niemann-Pick Type C1 (NPC1) or C2 (NPC2) Disease
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Title
Pulmonary Abnormalities in Animal Models Due to Niemann-Pick Type C1 (NPC1) or C2 (NPC2) Disease
Authors
Keywords
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Journal
PLoS One
Volume 8, Issue 7, Pages e67084
Publisher
Public Library of Science (PLoS)
Online
2013-07-03
DOI
10.1371/journal.pone.0067084
References
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Related references
Note: Only part of the references are listed.- Characterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lung
- (2012) Blair R. Roszell et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Free cholesterol accumulation impairs antioxidant activities and aggravates apoptotic cell death in menadione-induced oxidative injury
- (2011) Waisin Lee et al. ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
- A novel mouse model of Niemann–Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations
- (2011) Robert A. Maue et al. HUMAN MOLECULAR GENETICS
- A role for oxysterol-binding protein–related protein 5 in endosomal cholesterol trafficking
- (2011) Ximing Du et al. JOURNAL OF CELL BIOLOGY
- Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1−/− mice
- (2011) Akshay Muralidhar et al. MOLECULAR GENETICS AND METABOLISM
- Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding
- (2011) M. S. Deffieu et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Conditional deletion of Abca3 in alveolar type II cells alters surfactant homeostasis in newborn and adult mice
- (2010) Valérie Besnard et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion
- (2010) Misbaudeen Abdul-Hammed et al. JOURNAL OF LIPID RESEARCH
- Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches
- (2010) Anton I. Rosenbaum et al. JOURNAL OF NEUROCHEMISTRY
- 2-Hydroxypropyl-β-Cyclodextrin Raises Hearing Threshold in Normal Cats and in Cats With Niemann-Pick Type C Disease
- (2010) Sarah Ward et al. PEDIATRIC RESEARCH
- Weekly Cyclodextrin Administration Normalizes Cholesterol Metabolism in Nearly Every Organ of the Niemann-Pick Type C1 Mouse and Markedly Prolongs Life
- (2010) Charina M Ramirez et al. PEDIATRIC RESEARCH
- Role of cholesterol in the biophysical dysfunction of surfactant in ventilator-induced lung injury
- (2009) Dan Vockeroth et al. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
- Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol
- (2009) Hyock Joo Kwon et al. CELL
- Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis
- (2009) M Griese et al. CLINICAL GENETICS
- Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid
- (2009) Benny Liu et al. JOURNAL OF LIPID RESEARCH
- Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span ofNpc1-/-mice
- (2008) Min Zhang et al. JOURNAL OF NEUROSCIENCE RESEARCH
- NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes
- (2008) R. E. Infante et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
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