Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C. - Wikidata - awgldk - TriplyDB

Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

http://www.wikidata.org/entity/Q34544916

about

Niemann-Pick disease type C Regulation of sterol transport between membranes and NPC2 Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect.Niemann-Pick type C disease and intracellular cholesterol trafficking.[International Children's Day]Misdiagnosis of Niemann-Pick disease type C as Gaucher disease Secondary lipid accumulation in lysosomal disease.Acidic Ca2+ stores in neurodegeneration.ALCAT1 is a polyglycerophospholipid acyltransferase potently regulated by adenine nucleotide and thyroid status Partial blockage of sterol biosynthesis with a squalene synthase inhibitor in early postnatal Niemann-Pick type C npcnih null mice brains reduces neuronal cholesterol accumulation, abrogates astrogliosis, but may inhibit myelin maturation.Visualization of cholesterol deposits in lysosomes of Niemann-Pick type C fibroblasts using recombinant perfringolysin O.Complex lipid trafficking in Niemann-Pick disease type C.A novel function of the human CLS1 in phosphatidylglycerol synthesis and remodeling.The N370S (Asn370-->Ser) mutation affects the capacity of glucosylceramidase to interact with anionic phospholipid-containing membranes and saposin C N370S-GBA1 mutation causes lysosomal cholesterol accumulation in Parkinson's disease.The lipoprotein receptor-related protein-1 (LRP) adapter protein GULP mediates trafficking of the LRP ligand prosaposin, leading to sphingolipid and free cholesterol accumulation in late endosomes and impaired efflux.The importance of cholesterol in Parkinson's disease.

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P2860

Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

http://www.wikidata.org/entity/Q34544916

description

2004 nî lūn-bûn

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2004 թուականի Փետրուարին հրատարակուած գիտական յօդուած

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2004 թվականի փետրվարին հրատարակված գիտական հոդված

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2004年の論文

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2004年論文

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2004年論文

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2004年論文

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2004年論文

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2004年論文

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2004年论文

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name

Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

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P1433

Journal of Biological Chemistry

P1476

Glucosylceramidase mass and su ...... n Niemann-Pick disease type C.

@en

P2093

Anna Maria Vaccaro

http://www.w3.org/2001/XMLSchema#string

Carlo Ramoni

http://www.w3.org/2001/XMLSchema#string

Fiorella Ciaffoni

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Marie T Vanier

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Massimo Tatti

http://www.w3.org/2001/XMLSchema#string

Rosa Salvioli

http://www.w3.org/2001/XMLSchema#string

Susanna Scarpa

http://www.w3.org/2001/XMLSchema#string

P2860

Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-acti

Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis

Niemann-Pick C1 disease: correlations between NPC1 mutations, levels of NPC1 protein, and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop.

Cleavage of structural proteins during the assembly of the head of bacteriophage T4

Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids

Sterol-modulated glycolipid sorting occurs in niemann-pick C1 late endosomes.

Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature.

The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo.

Subcellular targeting by membrane lipids.

P304

17674-17680

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scholarly article

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10.1074/JBC.M313517200

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P433

17

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P478

279

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2004-02-02T00:00:00Z

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P698

14757764

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