Results 191 to 200 of about 16,701 (225)

Deficiency of Niemann‐Pick C1 protein protects against diet‐induced gallstone formation in mice

Liver international : official journal of the International Association for the Study of the Liver, 2010
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M. Morales, L. Amigo, Elisa Balboa, Mariana Acuña, J. Castro, H. Molina, J. Miquel, F. Nervi, A. Rigotti, S. Zanlungo   +9 more
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Transport of plasma membrane‐derived cholesterol and the function of Niemann‐Pick C1 protein

The FASEB Journal, 2003
ABSTRACT To visualize the intracellular transport of plasma membrane‐derived cholesterol under physiological and pathophysiological conditions, a novel fluorescent cholesterol analog, 6‐dansyl cholestanol (DChol), has been synthesized. We present several lines of evidence that DChol mimics cholesterol.
Volker Wiegand, Ta-Yuan Chang, J. Strauss, F. Fahrenholz, G. Gimpl   +4 more
semanticscholar   +3 more sources

mdr1a deficiency corrects sterility in Niemann–Pick C1 protein deficient female mice

Molecular Reproduction and Development, 2002
AbstractNiemann–Pick type C disease is a progressive neurological disease with cholesterol storage in liver, and npc1−/− mice share these features and are sterile. We have searched for the cause of sterility and found normal folliculogenesis and progesterone levels but lack of implantation.
R. Erickson, M. Kiela, P. Devine, P. Hoyer, R. Heidenreich   +4 more
semanticscholar   +3 more sources

Niemann-Pick C1 protein regulates platelet membrane-associated Ca2+ signalling in thrombo-occlusive diseases in mice.

Journal of Thrombosis and Haemostasis, 2023
BACKGROUND Pathophysiological platelet activation leads to thrombo-occlusive diseases such as myocardial infarction or ischemic stroke. Niemann-Pick C1 protein (NPC1) is involved in the regulation of lysosomal lipid trafficking and Ca2+ signalling and ...
M. Manke, A. Roslan, B. Walker, P. Münzer, F. Kollotzek, B. Peng, S. Mencl, C. Coman, R. Szepanowski, H. Schulze, A. Lieberman, F. Lang, M. Gawaz, C. Kleinschnitz, R. Lukowski, R. Ahrends, R. Bobe, O. Borst   +17 more
semanticscholar   +1 more source

Examining the Role of Niemann-Pick C1 Protein in the Permissiveness of Aedes Mosquitoes to Filoviruses.

ACS Infectious Diseases, 2020
Aedes mosquitoes vector many viruses with divergent characteristics, yet the criteria needed for a virus to be vectored by an arthropod remain unknown.
Alexander S. Gold, Fabiana Feitosa-Suntheimer, S. Asad, Bukola Adeoye, J. Connor, T. Colpitts   +5 more
semanticscholar   +1 more source

Niemann-Pick C1 Like 1 Protein Is Critical for Intestinal Cholesterol Absorption

Science, 2004
Dietary cholesterol consumption and intestinal cholesterol absorption contribute to plasma cholesterol levels, a risk factor for coronary heart disease. The molecular mechanism of sterol uptake from the lumen of the small intestine is poorly defined.
Scott W, Altmann, Harry R, Davis, Li-Ji, Zhu, Xiaorui, Yao, Lizbeth M, Hoos, Glen, Tetzloff, Sai Prasad N, Iyer, Maureen, Maguire, Andrei, Golovko, Ming, Zeng, Luquan, Wang, Nicholas, Murgolo, Michael P, Graziano   +12 more
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Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides.

Experimental Cell Research, 2009
Wilson disease is a genetic disorder characterized by the accumulation of copper in the body by defective biliary copper excretion. Wilson disease gene product (ATP7B) functions in copper incorporation to ceruloplasmin (Cp) and biliary copper excretion. However, copper metabolism in hepatocytes has been still unclear.
C. Yanagimoto, M. Harada, Hiroto Kumemura, H. Koga, T. Kawaguchi, K. Terada, S. Hanada, E. Taniguchi, Yukio Koizumi, S. Koyota, H. Ninomiya, T. Ueno, T. Sugiyama, M. Sata   +13 more
semanticscholar   +3 more sources

Exploring sterol transportation behavior of the Niemann-Pick C1-like 1 protein with V55L mutation: Sterol-NPC1L1 N-terminal binding energy estimation via molecular dynamics simulations

International Journal of Modelling and Simulation, 2023
The Niemann-Pick C1-like 1 (NPC1L1) protein facilitates cholesterol absorption in the small intestine and mediates the absorption of other sterols, including vitamins E, vitamin K1, and coenzyme Q10 (CoQ10).
Hye-Jin Yoon, Yeeun Lee, Sun-hong Kim, Eunae Kim, Hyung Ho Lee, S. Jang   +5 more
semanticscholar   +1 more source

The structure and function of Niemann–Pick C1-like 1 protein

Current Opinion in Lipidology, 2008
Intestinal absorption and biliary excretion of cholesterol represent two major pathways by which the body regulates cholesterol homeostasis. Niemann-Pick C1-like 1 (NPC1L1) is a polytopic transmembrane protein containing a sterol-sensing domain of unknown function.
openaire   +2 more sources

Structure–activity relationships of oxysterol-derived pharmacological chaperones for Niemann–Pick type C1 protein

Bioorganic & Medicinal Chemistry Letters, 2014
Niemann-Pick disease type C is a fatal neurodegenerative disease, and its major cause is mutations in NPC1 gene. This gene encodes NPC1 protein, a late endosomal polytopic membrane protein required for intracellular cholesterol trafficking. One prevalent mutation (I1061T) has been shown to cause a folding defect, which results in failure of endosomal ...
Kenji, Ohgane, Fumika, Karaki, Tomomi, Noguchi-Yachide, Kosuke, Dodo, Yuichi, Hashimoto   +4 more
openaire   +2 more sources