Cholesterol binding to the sterol-sensing region of Niemann Pick C1 protein confines dynamics of its N-terminal domain. [PDF]
PLoS Computational Biology, 2020 Lysosomal accumulation of cholesterol is a hallmark of Niemann Pick type C (NPC) disease caused by mutations primarily in the lysosomal membrane protein NPC1.
Vikas Dubey +3 more
doaj +3 more sources
Inhibition of the Niemann-Pick C1 protein is a conserved feature of multiple strains of pathogenic mycobacteria [PDF]
Nature Communications, 2022 Lipids shed by pathogenic mycobacteria have been shown to inhibit NPC1, a lysosomal membrane protein deficient in most cases of a rate inherited lysosomal storage disorder Niemann-Pick disease type C (NPC).
Yuzhe Weng +7 more
doaj +3 more sources
Identification of Niemann-Pick C1 protein as a potential novel SARS-CoV-2 intracellular target. [PDF]
Antiviral Res, 2021 Niemann-Pick type C1 (NPC1) receptor is an endosomal membrane protein that regulates intracellular cholesterol traffic. This protein has been shown to play an important role for several viruses.
García-Dorival I +11 more
europepmc +2 more sources
Purification and structure of luminal domain C of human Niemann-Pick C1 protein. [PDF]
Acta Crystallogr F Struct Biol Commun, 2023 New purification protocols for the second luminal domain of Nieman–Pick C1 protein, which serves as the intracellular receptor for Ebola and Marburg viruses, are reported together with a crystal structure, offering a structural view of the Ebola virus ...
Odongo L +7 more
europepmc +2 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 Niemann-Pick C1 Protein of Patients with Hepatocellular Carcinoma Is Associated with Survival Time in Males and Tumor Size in Females. [PDF]
BiomedicinesBackground/Objectives: The Niemann–Pick C1 (NPC1) protein regulates cellular cholesterol homeostasis, which is disrupted in hepatocellular carcinoma (HCC). Sex differences in cholesterol metabolism may also be related to NPC1 expression in HCC.
Weber F +5 more
europepmc +2 more sources
Differential Trafficking Phenotypes of NPC1 Mutant Proteins Reveal Distinct Cholesterol Accumulation Profiles. [PDF]
J Inherit Metab DisABSTRACT Niemann‐Pick disease type C (NPC) is a rare autosomal recessive lysosomal storage disorder that affects approximately 1 in 100 000 live births. It is primarily caused by mutations in the NPC1 gene, which disrupts intracellular cholesterol transport and leads to lipid accumulation in late endosomes and lysosomes.
Mahmoud SA +3 more
europepmc +2 more sources
The Niemann-Pick C1 protein in recycling endosomes of presynaptic nerve terminals
Journal of Lipid Research, 2006 Niemann-Pick type C (NPC) disease is a fatal, neurodegenerative disorder caused in 95% of cases by loss of function of NPC1, a ubiquitous endosomal transmembrane protein.
Barbara Karten +3 more
doaj +2 more sources
Cell Communication and Signaling, 2023 Viruses communicate with their hosts through interactions with proteins, lipids, and carbohydrate moieties on the plasma membrane (PM), often resulting in viral absorption via receptor-mediated endocytosis.
Irfan Ahmad +8 more
doaj +2 more sources