Niemann-Pick C1 Heterogeneity of Bat Cells Controls Filovirus Tropism [PDF]
Cell Reports, 2020 Summary: Fruit bats are suspected to be natural hosts of filoviruses, including Ebola virus (EBOV) and Marburg virus (MARV). Interestingly, however, previous studies suggest that these viruses have different tropisms depending on the bat species.
Yoshihiro Takadate +15 more
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Lysosomal cholesterol export reconstituted from fragments of Niemann-Pick C1 [PDF]
eLife, 2018 Niemann-Pick C1 (NPC1) is a polytopic membrane protein with 13 transmembrane helices that exports LDL-derived cholesterol from lysosomes by carrying it through the 80 Å glycocalyx and the 40 Å lipid bilayer. Transport begins when cholesterol binds to the
Michael Nguyen Trinh +4 more
doaj +4 more sources
Defective platelet function in Niemann‐Pick disease type C1 [PDF]
JIMD Reports, 2020 Niemann‐Pick disease type C (NPC) is a neurodegenerative lysosomal storage disorder caused by mutations in either NPC1 (95% of cases) or NPC2. Reduced late endosome/lysosome calcium (Ca2+) levels and the accumulation of unesterified cholesterol and ...
Oscar C. W. Chen +13 more
doaj +5 more sources
Adenosine A2A Receptor Activation Regulates Niemann–Pick C1 Expression and Localization in Macrophages [PDF]
Current Issues in Molecular Biology, 2023 Adenosine plays an important role in modulating immune cell function, particularly T cells and myeloid cells, such as macrophages and dendritic cells. Cell surface adenosine A2A receptors (A2AR) regulate the production of pro-inflammatory cytokines and ...
Adrienn Skopál +8 more
doaj +2 more sources
Intestinal and Hepatic Niemann-Pick C1-Like 1 [PDF]
Diabetes & Metabolism Journal, 2013 Polytopic transmembrane protein, Niemann-Pick C1-Like 1 (NPC1L1) is localized at the apical membrane of enterocytes and the canalicular membrane of hepatocytes.
Sung-Woo Park
doaj +4 more sources
Niemann-Pick C1 (NPC1)/NPC1-like1 Chimeras Define Sequences Critical for NPC1’s Function as a Filovirus Entry Receptor [PDF]
Viruses, 2012 We recently demonstrated that Niemann-Pick C1 (NPC1), a ubiquitous 13-pass cellular membrane protein involved in lysosomal cholesterol transport, is a critical entry receptor for filoviruses.
Esther Ndungo +7 more
doaj +4 more sources
Quantitating the epigenetic transformation contributing to cholesterol homeostasis using Gaussian process [PDF]
Nature Communications, 2019 How epigenetics coordinate with genetics to impact protein fitness is unknown. Here, using a Variation Spatial Profiling strategy and machine learning, the authors map HDAC impact on a full set of Niemann pick C1 disease variants to quantitate an ...
Chao Wang +8 more
doaj +3 more sources
The Cerebellum in Niemann-Pick C1 Disease: Mouse Versus Man. [PDF]
Cerebellum, 2023 Selective neuronal vulnerability is common to most degenerative disorders, including Niemann-Pick C (NPC), a rare genetic disease with altered intracellular trafficking of cholesterol. Purkinje cell dysfunction and loss are responsible for cerebellar ataxia, which is among the prevailing neurological signs of the NPC disease.
Fiorenza MT +3 more
europepmc +3 more sources
A Niemann‐pick C1 disease child with BCG-itis: a case report and analysis [PDF]
BMC Pediatrics, 2021 Background Niemann-Pick C disease is a rare autosomal recessive lysosomal lipid storage disorder. Some primary immunodeficiency diseases patients developed regional disease or disseminated disease after vaccinating BCG.
Jing-jing Lin +5 more
doaj +2 more sources
Structure of human Niemann-Pick C1 protein. [PDF]
Proc Natl Acad Sci U S A, 2016 Significance Niemann–Pick C1 protein (NPC1) is a late-endosomal membrane protein required for transport of LDL-derived cholesterol into cells and Ebola virus entry; mutations cause Niemann–Pick type C disease. NPC1 contains a “sterol-sensing domain” (SSD) that also appears in several key regulatory proteins of cholesterol biosynthesis,
Li X +5 more
europepmc +4 more sources