Results 101 to 110 of about 15,838 (212)

First data on cholesterol metabolism in Ornithodoros argasid ticks: Molecular and functional characterization of the N-terminal domain of Niemann-Pick C1 proteins

Ticks and Tick-Borne Diseases
Cholesterol is a molecule vital for tick physiology, but ticks cannot synthesize it and rely on dietary cholesterol. Therefore, tick proteins involved in cholesterol absorption and transport, such as the Niemann-Pick type C1 domain-containing (NPC1 ...
Lucía de Dios-Blázquez, Ana Laura Cano-Argüelles, Ricardo Pérez-Sánchez, María González-Sánchez, Ana Oleaga   +4 more
doaj   +1 more source

A human neuronal model of Niemann Pick C disease developed from stem cells isolated from patient's skin. [PDF]

, 2013
Niemann Pick C (NPC) disease is a neurovisceral lysosomal storage disorder due to mutations in NPC1 or NPC2 genes, characterized by the accumulation of endocytosed unesterified cholesterol, gangliosides and other lipids within the lysosomes/late ...
Beltrami, Antonio Paolo, Beltrami, Carlo Alberto, Bembi, B, Bergamin, N, Cesselli, Daniela, Dardis, A, Domenis, Rossana, Rigo, S, Zampieri, S   +8 more
core   +2 more sources

Recovery from liver disease in a Niemann-Pick type C mouse model

Journal of Lipid Research, 2010
Loss of function of Niemann-Pick C1 (NPC1) leads to lysosomal free cholesterol storage, resulting in the neurodegenerative disease Niemann-Pick disease type C (NPC). Significant numbers of patients with NPC also suffer from liver disease.
Naomi L. Sayre, Victoria M. Rimkunas, Mark J. Graham, Rosanne M. Crooke, Laura Liscum   +4 more
doaj   +1 more source

Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations

Journal of Lipid Research, 2011
Lipoprotein cholesterol taken up by cells is processed in the endosomal/lysosomal (E/L) compartment by the sequential action of lysosomal acid lipase (LAL), Niemann-Pick C2 (NPC2), and Niemann-Pick C1 (NPC1).
Charina M. Ramirez, Benny Liu, Amal Aqul, Anna M. Taylor, Joyce J. Repa, Stephen D. Turley, John M. Dietschy   +6 more
doaj   +1 more source

Psychosis in an adolescent girl: a common manifestation in Niemann-Pick Type C disease [PDF]

, 2014
Niemann-Pick disease type C (NP-C) is a rare autosomal-recessively inherited lysosomal storage disorder. It is caused by mutations in the NPC1 (95%) or NPC2 gene.
Annik Lampo, Edward Campforts, Linda De Meirleir, Sara Wouters   +3 more
core   +1 more source

Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

Cell Reports, 2013
Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear.
Sovan Sarkar, Bernadette Carroll, Yosef Buganim, Dorothea Maetzel, Alex H.M. Ng, John P. Cassady, Malkiel A. Cohen, Souvik Chakraborty, Haoyi Wang, Eric Spooner, Hidde Ploegh, Joerg Gsponer, Viktor I. Korolchuk, Rudolf Jaenisch   +13 more
doaj   +1 more source

Efficiency of siRNA delivery by lipid nanoparticles is limited by endocytic recycling [PDF]

, 2013
Despite substantial efforts to understand the interactions between nanoparticles and cells, the cellular processes that determine the efficiency of intracellular drug delivery remain largely unclear. Here we examined cellular uptake of siRNA delivered in
A Akinc, Ahmed Eltoukhy, Avi Schroeder, B Ravikumar, C Collinet, C Sheridan, CA Alabi, Christopher Alabi, Christopher Zurenko, D Chen, Daniel G Anderson, DC Ko, Delai Chen, DJ Gibbings, E Ikonen, Emmanouil Karagiannis, F Leuschner, G Basha, G Sahay, Gaurav Sahay, GJ Doherty, H Siomi, H Stenmark, IA Khalil, J Nguyen, J Suh, J Wang, JE Carette, JJ Lu, KA Whitehead, Kevin Love, KT Love, L Stalder, M Huss, M Hölttä-Vuori, M Koivusalo, M Ostrowski, M Zhang, MD Linder, ME Davis, N Bayer, ND Sonawane, P Shannon, R Zoncu, RL Juliano, Robert Langer, Roberto Zoncu, S Maere, S Sarkar, SK Lai, SM Elbashir, Sovan Sarkar, William Querbes, Yosef Buganim, YS Lee   +54 more
core   +2 more sources

Interaction of cholesterol with sphingosine

Journal of Lipid Research, 2005
Molecular associations between sphingomyelin and cholesterol provide a molecular basis for the colocalization of these lipids in plasma membrane microdomains (lipid rafts) and for the inhibitory effect of sphingomyelin on the intestinal absorption of ...
Nicolas Garmy, Nadira Taïeb, Nouara Yahi, Jacques Fantini   +3 more
doaj   +1 more source

Purification and structure of luminal domain C of human Niemann-Pick C1 protein. [PDF]

Acta Crystallogr F Struct Biol Commun, 2023
Odongo L, Zadrozny KK, Diehl WE, Luban J, White JM, Ganser-Pornillos BK, Tamm LK, Pornillos O.   +7 more
europepmc   +1 more source

Development of a neutralization assay and bioluminescent imaging mouse model for Dehong virus (DEHV) using a pseudovirus system

Microbiology Spectrum
Dehong virus (DEHV) is an emerging filamentous virus of considerable interest. However, research involving DEHV remains limited, and no suitable models exist to investigate its pathogenicity or transmission.
Xuelian Wu, Fan Liu, Tao Li, Danfeng Li, Yanru Shen, Xiaoai Zhang, Shuo Liu, Qi Jiang, Chenyan Zhao, Jianhui Nie, Youchun Wang, Baomin Feng, Wei Liu, Weijin Huang   +13 more
doaj   +1 more source