The integrity of a cholesterol-binding pocket in Niemann-Pick C2 protein is necessary to control lysosome cholesterol levels.
about
Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progressionNovel function of Niemann-Pick C1-like 1 as a negative regulator of Niemann-Pick C2 proteinNPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterolsNPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterolsNPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomesNogo-B receptor stabilizes Niemann-Pick type C2 protein and regulates intracellular cholesterol traffickingRegulation of sterol transport between membranes and NPC2Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 diseaseCholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cellsThe pathogenesis of Niemann-Pick type C disease: a role for autophagy?Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C diseaseGenetic and laboratory diagnostic approach in Niemann Pick disease type CStructural Basis of Sterol Binding by NPC2, a Lysosomal Protein Deficient in Niemann-Pick Type C2 DiseaseStructure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of CholesterolSaccharomyces cerevisiae Npc2p is a functionally conserved homologue of the human Niemann-Pick disease type C 2 protein, hNPC2.Vasectomy influences expression of HE1 but not HE2 and HE5 genes in human epididymisMLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 proteinGenetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transportThe Niemann-Pick C1 and caveolin-1 proteins interact to modulate efflux of low density lipoprotein-derived cholesterol from late endocytic compartments.ProPhylER: a curated online resource for protein function and structure based on evolutionary constraint analyses.Adaptations of energy metabolism associated with increased levels of mitochondrial cholesterol in Niemann-Pick type C1-deficient cells.Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Early steps in steroidogenesis: intracellular cholesterol trafficking.Glycine N-methyltransferase deficiency affects Niemann-Pick type C2 protein stability and regulates hepatic cholesterol homeostasis.Niemann-Pick type C disease and intracellular cholesterol trafficking.Somatic cell plasticity and Niemann-Pick type C2 protein: fibroblast activation.Identification of surface residues on Niemann-Pick C2 essential for hydrophobic handoff of cholesterol to NPC1 in lysosomesNiemann-Pick type C pathogenesis and treatment: from statins to sugars.Lipid rafts: keys to sperm maturation, fertilization, and early embryogenesis.An "exacerbate-reverse" strategy in yeast identifies histone deacetylase inhibition as a correction for cholesterol and sphingolipid transport defects in human Niemann-Pick type C disease.Potential pitfalls and solutions for use of fluorescent fusion proteins to study the lysosomeAge and prior blood feeding of Anopheles gambiae influences their susceptibility and gene expression patterns to ivermectin-containing blood mealsSterol transport in yeast and the oxysterol binding protein homologue (OSH) familyProteomic analysis of Schistosoma mansoni egg secretions.Non-vesicular sterol transport in cells.Lipid imbalance in the neurological disorder, Niemann-Pick C disease.Mechanisms for cellular cholesterol transport: defects and human disease.Transport of LDL-derived cholesterol from the NPC1 compartment to the ER involves the trans-Golgi network and the SNARE protein complexNiemann-pick type C2 deficiency in human fibroblasts confers robust and selective activation of prostaglandin E2 biosynthesis.Niemann-Pick Type C2 Protein Mediates Hepatic Stellate Cells Activation by Regulating Free Cholesterol Accumulation
P2860
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P2860
The integrity of a cholesterol-binding pocket in Niemann-Pick C2 protein is necessary to control lysosome cholesterol levels.
description
2003 nî lūn-bûn
@nan
2003 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@ast
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@en
type
label
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@ast
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@en
prefLabel
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@ast
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@en
P2093
P2860
P356
P1476
The integrity of a cholesterol ...... l lysosome cholesterol levels.
@en
P2093
Arend Sidow
Dennis C Ko
Jonathan Binkley
Matthew P Scott
P2860
P304
P356
10.1073/PNAS.0530027100
P407
P577
2003-02-18T00:00:00Z