Delineation of metabolic responses of Npc1 mice lacking the cholesterol-esterifying enzyme SOAT2 to acute treatment with 2-hydroxypropyl-β-cyclodextrin
Published 2020 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
Delineation of metabolic responses of Npc1 mice lacking the cholesterol-esterifying enzyme SOAT2 to acute treatment with 2-hydroxypropyl-β-cyclodextrin
Authors
Keywords
Lysosomal storage disease, Unesterified cholesterol sequestration, Cholesterol synthesis and esterification, Liver, Spleen
Journal
STEROIDS
Volume 164, Issue -, Pages 108725
Publisher
Elsevier BV
Online
2020-09-02
DOI
10.1016/j.steroids.2020.108725
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1
- (2020) Jorge L. Rodriguez-Gil et al. Disease Models & Mechanisms
- Animal models for Niemann-Pick type C: implications for drug discovery & development
- (2019) Cathrine K. Fog et al. Expert Opinion on Drug Discovery
- Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann–Pick C1 Disease
- (2019) Adam M. Lopez et al. DIGESTIVE DISEASES AND SCIENCES
- Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function
- (2018) Adam M. Lopez et al. AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
- Long term substrate reduction therapy with ezetimibe alone or associated with statins in three adult patients with lysosomal acid lipase deficiency
- (2018) Maja Di Rocco et al. Orphanet Journal of Rare Diseases
- Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse
- (2018) Adam M. Lopez et al. STEROIDS
- Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1–2 trial
- (2017) Daniel S Ory et al. LANCET
- Cyclodextrins as Emerging Therapeutic Tools in the Treatment of Cholesterol-Associated Vascular and Neurodegenerative Diseases
- (2016) Caroline Coisne et al. MOLECULES
- A Murine Niemann-Pick C1 I1061T Knock-In Model Recapitulates the Pathological Features of the Most Prevalent Human Disease Allele
- (2015) M. Praggastis et al. JOURNAL OF NEUROSCIENCE
- Efficacy and Safety of Ezetimibe Monotherapy in Children with Heterozygous Familial or Nonfamilial Hypercholesterolemia
- (2015) D. Meeike Kusters et al. JOURNAL OF PEDIATRICS
- Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease
- (2015) Charles H. Vite et al. Science Translational Medicine
- Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function
- (2014) Adam M Lopez et al. CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY
- Collaborative Development of 2-Hydroxypropyl-β-Cyclodextrin for the Treatment of Niemann-Pick Type C1 Disease
- (2014) Elizabeth Ottinger et al. CURRENT TOPICS IN MEDICINAL CHEMISTRY
- Molecular Mechanisms of Cellular Cholesterol Efflux
- (2014) Michael C. Phillips JOURNAL OF BIOLOGICAL CHEMISTRY
- Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin
- (2014) Jean E. Vance et al. JOURNAL OF LIPID RESEARCH
- Hepatic ACAT2 Knock Down Increases ABCA1 and Modifies HDL Metabolism in Mice
- (2014) Matteo Pedrelli et al. PLoS One
- Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease
- (2013) Jen-Chieh Chuang et al. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
- Controlling Cholesterol Synthesis beyond 3-Hydroxy-3-methylglutaryl-CoA Reductase (HMGCR)
- (2013) Laura J. Sharpe et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Lung toxicity of hydroxypropyl-β-cyclodextrin infusion
- (2013) Yin-Hsiu Chien et al. MOLECULAR GENETICS AND METABOLISM
- Therapeutic potential of cyclodextrins in the treatment of Niemann–Pick type C disease
- (2012) Benny Liu Clinical Lipidology
- Cholesteryl esters and ACAT
- (2012) Camilla Pramfalk et al. EUROPEAN JOURNAL OF LIPID SCIENCE AND TECHNOLOGY
- Lecithin:cholesterol acyltransferase: old friend or foe in atherosclerosis?
- (2012) Sandra Kunnen et al. JOURNAL OF LIPID RESEARCH
- Cyclodextrin mediates rapid changes in lipid balance inNpc1−/−mice without carrying cholesterol through the bloodstream
- (2012) Anna M. Taylor et al. JOURNAL OF LIPID RESEARCH
- Effects of cyclodextrin in two patients with Niemann–Pick Type C disease
- (2012) Muneaki Matsuo et al. MOLECULAR GENETICS AND METABOLISM
- 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
- Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations
- (2011) Charina M. Ramirez et al. JOURNAL OF LIPID RESEARCH
- Unesterified Cholesterol Accumulation in Late Endosomes/Lysosomes Causes Neurodegeneration and Is Prevented by Driving Cholesterol Export from This Compartment
- (2011) A. Aqul et al. JOURNAL OF NEUROSCIENCE
- Defective cholesterol trafficking in Niemann-Pick C-deficient cells
- (2010) Kyle B. Peake et al. FEBS LETTERS
- Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches
- (2010) Anton I. Rosenbaum et al. JOURNAL OF NEUROCHEMISTRY
- Niemann-Pick disease type C
- (2010) Marie T Vanier Orphanet Journal of Rare Diseases
- Niemann–Pick C1 Like 1 (NPC1L1) an intestinal sterol transporter
- (2009) Harry R. Davis et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
- Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol
- (2009) Hyock Joo Kwon et al. CELL
- 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
- Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression
- (2009) Cristin D. Davidson et al. PLoS One
- Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse
- (2009) B. Liu et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Find Funding. Review Successful Grants.
Explore over 25,000 new funding opportunities and over 6,000,000 successful grants.
ExploreDiscover Peeref hubs
Discuss science. Find collaborators. Network.
Join a conversation