Publications by authors named "Stephen D Turley"

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Molecular markers of brain cholesterol homeostasis are unchanged despite a smaller brain mass in a mouse model of cholesteryl ester storage disease.
Amal A Aqul, Charina M Ramirez, Adam M Lopez, Dennis K Burns, Joyce J Repa, Stephen D Turley

Lipids· January 2022

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Delineation of metabolic responses of Npc1 mice lacking the cholesterol-esterifying enzyme SOAT2 to acute treatment with 2-hydroxypropyl-β-cyclodextrin.
Charina M Ramirez, Anna M Taylor, Adam M Lopez, Joyce J Repa, Stephen D Turley

Steroids· December 2020

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Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann-Pick C1 Disease.
Adam M Lopez, Charina M Ramirez, Anna M Taylor, Ryan D Jones, Joyce J Repa, Stephen D Turley

Dig Dis Sci· January 2020

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Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function.
Adam M Lopez, Ryan D Jones, Joyce J Repa, Stephen D Turley

Am J Physiol Gastrointest Liver Physiol· October 2018

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Identification of Correlative Shifts in Indices of Brain Cholesterol Metabolism in the C57BL6/Mecp2 Mouse, a Model for Rett Syndrome.
Dieter Lütjohann, Adam M Lopez, Jen-Chieh Chuang, Anja Kerksiek, Stephen D Turley

Lipids· April 2018

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Measurement of Rates of Cholesterol and Fatty Acid Synthesis In Vivo Using Tritiated Water.
Adam M Lopez, Jen-Chieh Chuang, Stephen D Turley

Methods Mol Biol· February 2018

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Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse.
Adam M Lopez, Jen-Chieh Chuang, Stephen D Turley

Steroids· February 2018

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Quantitation of the rates of hepatic and intestinal cholesterol synthesis in lysosomal acid lipase-deficient mice before and during treatment with ezetimibe.
Jen-Chieh Chuang, Adam M Lopez, Stephen D Turley

Biochem Pharmacol· July 2017

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'Corrigenda to "Suppression of brain cholesterol synthesis in male Mecp2-deficient mice is age dependent and not accompanied by a concurrent change in the rate of fatty acid synthesis" [Brain Res. 1654 (2017) 77-84]'.
Adam M Lopez, Jen-Chieh Chuang, Kenneth S Posey, Stephen D Turley

Brain Res· February 2017

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Suppression of brain cholesterol synthesis in male Mecp2-deficient mice is age dependent and not accompanied by a concurrent change in the rate of fatty acid synthesis.
Adam M Lopez, Jen-Chieh Chuang, Kenneth S Posey, Stephen D Turley

Brain Res· January 2017

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PRD125, a potent and selective inhibitor of sterol O-acyltransferase 2 markedly reduces hepatic cholesteryl ester accumulation and improves liver function in lysosomal acid lipase-deficient mice.
Adam M Lopez, Jen-Chieh Chuang, Kenneth S Posey, Taichi Ohshiro, Hiroshi Tomoda, Stephen D Turley

J Pharmacol Exp Ther· November 2015

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Lysosomal Acid Lipase Activity: A Tool for the Detection and Management of Fatty Liver Disease?
Charina M Ramirez, Adam M Lopez, Stephen D Turley

EBioMedicine· July 2015

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Impact of physiological levels of chenodeoxycholic acid supplementation on intestinal and hepatic bile acid and cholesterol metabolism in Cyp7a1-deficient mice.
Ryan D Jones, Adam M Lopez, Ernest Y Tong, Kenneth S Posey, Jen-Chieh Chuang, Stephen D Turley

Steroids· January 2015

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Deletion of sterol O-acyltransferase 2 (SOAT2) function in mice deficient in lysosomal acid lipase (LAL) dramatically reduces esterified cholesterol sequestration in the small intestine and liver.
Adam M Lopez, Kenneth S Posey, Stephen D Turley

Biochem Biophys Res Commun· November 2014

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Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function.
Adam M Lopez, Sandi J Terpack, Kenneth S Posey, Benny Liu, Charina M Ramirez, Stephen D Turley

Clin Exp Pharmacol Physiol· October 2014

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Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice.
Amal Aqul, Adam M Lopez, Kenneth S Posey, Anna M Taylor, Joyce J Repa, Stephen D Turley

Am J Physiol Gastrointest Liver Physiol· October 2014

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Impact of the loss of caveolin-1 on lung mass and cholesterol metabolism in mice with and without the lysosomal cholesterol transporter, Niemann-Pick type C1.
Dorothy I Mundy, Adam M Lopez, Kenneth S Posey, Jen-Chieh Chuang, Charina M Ramirez, Stephen D Turley

Biochim Biophys Acta· July 2014

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Sustained and selective suppression of intestinal cholesterol synthesis by Ro 48-8071, an inhibitor of 2,3-oxidosqualene:lanosterol cyclase, in the BALB/c mouse.
Jen-Chieh Chuang, Mark A Valasek, Adam M Lopez, Kenneth S Posey, Joyce J Repa, Stephen D Turley

Biochem Pharmacol· April 2014

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Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease.
Charina M Ramirez, Adam M Lopez, Lam Q Le, Kenneth S Posey, Arthur G Weinberg, Stephen D Turley

Biochim Biophys Acta· January 2014

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Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease.
Jen-Chieh Chuang, Adam M Lopez, Kenneth S Posey, Stephen D Turley

Biochem Biophys Res Commun· January 2014

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A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome.
Christie M Buchovecky, Stephen D Turley, Hannah M Brown, Stephanie M Kyle, Jeffrey G McDonald

Nat Genet· September 2013

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Delineation of biochemical, molecular, and physiological changes accompanying bile acid pool size restoration in Cyp7a1(-/-) mice fed low levels of cholic acid.
Ryan D Jones, Joyce J Repa, David W Russell, John M Dietschy, Stephen D Turley

Am J Physiol Gastrointest Liver Physiol· July 2012

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Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.
Amal Aqul, Benny Liu, Charina M Ramirez, Andrew A Pieper, Sandi Jo Estill, Stephen D Turley

J Neurosci· June 2011

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Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.
Charina M Ramirez, Benny Liu, Amal Aqul, Anna M Taylor, Joyce J Repa, Stephen D Turley

J Lipid Res· April 2011

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Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1.
Stephen D Turley, Mark A Valasek, Joyce J Repa, John M Dietschy

Am J Physiol Gastrointest Liver Physiol· November 2010

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