Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1⁻/⁻ mouse
Liu, Benny, Turley, Stephen D, Burns, Dennis K, Miller, Anna M, Repa, Joyce J, Dietschy, John M
Published in Proceedings of the National Academy of Sciences - PNAS (17.02.2009)
Published in Proceedings of the National Academy of Sciences - PNAS (17.02.2009)
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Journal Article
A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome
Buchovecky, Christie M, Turley, Stephen D, Brown, Hannah M, Kyle, Stephanie M, McDonald, Jeffrey G, Liu, Benny, Pieper, Andrew A, Huang, Wenhui, Katz, David M, Russell, David W, Shendure, Jay, Justice, Monica J
Published in Nature genetics (01.09.2013)
Published in Nature genetics (01.09.2013)
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In memoriam: John M. Dietschy Sr., MD (1932–2020)
Turley, Stephen D., Horton, Jay D., Andersen, John M., Fordtran, John S.
Published in Journal of lipid research (2021)
Published in Journal of lipid research (2021)
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Journal Article
Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid
Liu, Benny, Ramirez, Charina M., Miller, Anna M., Repa, Joyce J., Turley, Stephen D., Dietschy, John M.
Published in Journal of lipid research (01.05.2010)
Published in Journal of lipid research (01.05.2010)
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Journal Article
Weekly Cyclodextrin Administration Normalizes Cholesterol Metabolism in Nearly Every Organ of the Niemann-Pick Type C1 Mouse and Markedly Prolongs Life
RAMIREZ, Charina M, LIU, Benny, TAYLOR, Anna M, REPA, Joyce J, BURNS, Dennis K, WEINBERG, Arthur G, TURLEY, Stephen D, DIETSCHY, John M
Published in Pediatric research (01.10.2010)
Published in Pediatric research (01.10.2010)
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Journal Article
Genetic variations and treatments that affect the lifespan of the NPC1 mouse
Liu, Benny, Li, Hao, Repa, Joyce J., Turley, Stephen D., Dietschy, John M.
Published in Journal of lipid research (01.03.2008)
Published in Journal of lipid research (01.03.2008)
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Journal Article
Ontogenesis and Modulation of Intestinal Unesterified Cholesterol Sequestration in a Mouse Model of Niemann–Pick C1 Disease
Lopez, Adam M., Ramirez, Charina M., Taylor, Anna M., Jones, Ryan D., Repa, Joyce J., Turley, Stephen D.
Published in Digestive diseases and sciences (01.01.2020)
Published in Digestive diseases and sciences (01.01.2020)
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Journal Article
Liver X Receptor Activation Enhances Cholesterol Loss from the Brain, Decreases Neuroinflammation, and Increases Survival of the NPC1 Mouse
Repa, Joyce J, Li, Hao, Frank-Cannon, Tamy C, Valasek, Mark A, Turley, Stephen D, Tansey, Malu G, Dietschy, John M
Published in The Journal of neuroscience (26.12.2007)
Published in The Journal of neuroscience (26.12.2007)
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Journal Article
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
Lopez, Adam M, Posey, Kenneth S, Turley, Stephen D
Published in Biochemical and biophysical research communications (07.11.2014)
Published in Biochemical and biophysical research communications (07.11.2014)
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Journal Article
Knockout of the Cholesterol 24-Hydroxylase Gene in Mice Reveals a Brain-specific Mechanism of Cholesterol Turnover
Lund, Erik G, Xie, Chonglun, Kotti, Tiina, Turley, Stephen D, Dietschy, John M, Russell, David W
Published in The Journal of biological chemistry (20.06.2003)
Published in The Journal of biological chemistry (20.06.2003)
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Journal Article
Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations
Ramirez, Charina M., Liu, Benny, Aqul, Amal, Taylor, Anna M., Repa, Joyce J., Turley, Stephen D., Dietschy, John M.
Published in Journal of lipid research (01.04.2011)
Published in Journal of lipid research (01.04.2011)
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Journal Article
Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice
Aqul, Amal, Lopez, Adam M, Posey, Kenneth S, Taylor, Anna M, Repa, Joyce J, Burns, Dennis K, Turley, Stephen D
Published in American journal of physiology: Gastrointestinal and liver physiology (15.10.2014)
Published in American journal of physiology: Gastrointestinal and liver physiology (15.10.2014)
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Journal Article
Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease
Chuang, Jen-Chieh, Lopez, Adam M., Posey, Kenneth S., Turley, Stephen D.
Published in Biochemical and biophysical research communications (17.01.2014)
Published in Biochemical and biophysical research communications (17.01.2014)
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Journal Article
Inhibiting intestinal NPC1L1 activity prevents diet-induced increase in biliary cholesterol in Golden Syrian hamsters
Valasek, Mark A, Repa, Joyce J, Quan, Gang, Dietschy, John M, Turley, Stephen D
Published in American journal of physiology: Gastrointestinal and liver physiology (01.10.2008)
Published in American journal of physiology: Gastrointestinal and liver physiology (01.10.2008)
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Journal Article
Impact of physiological levels of chenodeoxycholic acid supplementation on intestinal and hepatic bile acid and cholesterol metabolism in Cyp7a1-deficient mice
Jones, Ryan D., Lopez, Adam M., Tong, Ernest Y., Posey, Kenneth S., Chuang, Jen-Chieh, Repa, Joyce J., Turley, Stephen D.
Published in Steroids (01.01.2015)
Published in Steroids (01.01.2015)
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Journal Article
Multiple mechanisms limit the accumulation of unesterified cholesterol in the small intestine of mice deficient in both ACAT2 and ABCA1
Turley, Stephen D, Valasek, Mark A, Repa, Joyce J, Dietschy, John M
Published in American journal of physiology: Gastrointestinal and liver physiology (01.11.2010)
Published in American journal of physiology: Gastrointestinal and liver physiology (01.11.2010)
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