Structural Basis of Sterol Binding by NPC2, a Lysosomal Protein Deficient in Niemann-Pick Type C2 Disease
about
NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomesRegulation of sterol transport between membranes and NPC2NPC1/NPC2 function as a tag team duo to mobilize cholesterolThe LDL receptorStructure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of CholesterolCrystal structure of soluble MD-1 and its interaction with lipid IVaNiemann-Pick type C2 protein mediating chemical communication in the worker antBinding domain-driven intracellular trafficking of sterols for synthesis of steroid hormones, bile acids and oxysterolsCharacterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lungLoss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathwayProtein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 diseaseNiemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 bindingEhNPC1 and EhNPC2 Proteins Participate in Trafficking of Exogenous Cholesterol in Entamoeba histolytica Trophozoites: Relevance for Phagocytosis.Proteins that bind and move lipids: MsbA and NPC1.Adaptations of energy metabolism associated with increased levels of mitochondrial cholesterol in Niemann-Pick type C1-deficient cells.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinCholesterol, the central lipid of mammalian cellsGenetic connections between neurological disorders and cholesterol metabolismTherapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Early steps in steroidogenesis: intracellular cholesterol trafficking.Scientific side trips: six excursions from the beaten path.Insights into the mechanisms of sterol transport between organelles.The carcinogenic liver fluke, Clonorchis sinensis: new assembly, reannotation and analysis of the genome and characterization of tissue transcriptomes.Characterization of a spontaneous novel mutation in the NPC2 gene in a cat affected by Niemann Pick type C disease.Identification of surface residues on Niemann-Pick C2 essential for hydrophobic handoff of cholesterol to NPC1 in lysosomesLipid rafts: keys to sperm maturation, fertilization, and early embryogenesis.Potential pitfalls and solutions for use of fluorescent fusion proteins to study the lysosomeThe N-terminal domain of NPC1L1 protein binds cholesterol and plays essential roles in cholesterol uptake.Amino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice.High incidence of unrecognized visceral/neurological late-onset Niemann-Pick disease, type C1, predicted by analysis of massively parallel sequencing data setsDesign and synthesis of a crosslinker for studying intracellular steroid trafficking pathways.Glycosylation inhibition reduces cholesterol accumulation in NPC1 protein-deficient cellsProteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.Clues to NPC1-mediated cholesterol export from lysosomes.Structure of human Niemann-Pick C1 protein.Lipid sorting and multivesicular endosome biogenesisIntracellular sterol dynamicsClues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2.Lysosomal membrane glycoproteins bind cholesterol and contribute to lysosomal cholesterol export.The potential of histone deacetylase inhibitors in Niemann - Pick type C disease.
P2860
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P2860
Structural Basis of Sterol Binding by NPC2, a Lysosomal Protein Deficient in Niemann-Pick Type C2 Disease
description
2007 nî lūn-bûn
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2007 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2007年の論文
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2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
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name
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@ast
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@en
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@nl
type
label
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@ast
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@en
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@nl
prefLabel
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@ast
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@en
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@nl
P2093
P2860
P3181
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P1476
Structural Basis of Sterol Bin ...... n Niemann-Pick Type C2 Disease
@en
P2093
Brian Benoff
Heng-Ling Liou
Peter Lobel
P2860
P304
P3181
P356
10.1074/JBC.M703848200
P407
P50
P577
2007-08-10T00:00:00Z