(Q30872586)

20 August 2017

Chemical screen to reduce sterol accumulation in Niemann-Pick C disease cells identifies novel lysosomal acid lipase inhibitors (English)

1 reference

Niemann–Pick disease, type C

20 August 2017

1 reference

Anton I Rosenbaum

1

1 reference

20 August 2017

Madalina Rujoi

2

1 reference

20 August 2017

Amy Y Huang

3

1 reference

20 August 2017

Hong Du

4

1 reference

20 August 2017

Gregory A Grabowski

5

1 reference

20 August 2017

Frederick R Maxfield

6

1 reference

20 August 2017

language of work or name

English

0 references

publication date

20 August 2009

1 reference

Biochimica et Biophysica Acta

20 August 2017

1 reference

20 August 2017

1791

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20 August 2017

12

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20 August 2017

1155-1165

1 reference

describes a project that uses

20 August 2017

ImageQuant

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC2783675/fullTextXML

inferred from PubMed Central ID database lookup

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Intracellular sterol dynamics

14 July 2018

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Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse

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Development of a Rab9 transgenic mouse and its ability to increase the lifespan of a murine model of Niemann-Pick type C disease

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14 July 2018

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Identification of neutral cholesterol ester hydrolase, a key enzyme removing cholesterol from macrophages

14 July 2018

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Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment

14 July 2018

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Stem cells in Niemann-Pick disease

14 July 2018

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Characterization of an exported monoglyceride lipase from Mycobacterium tuberculosis possibly involved in the metabolism of host cell membrane lipids

14 July 2018

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Treatment of Niemann-Pick disease type C in two children with miglustat: initial responses and maintenance of effects over 1 year.

14 July 2018

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Rab8-dependent recycling promotes endosomal cholesterol removal in normal and sphingolipidosis cells

14 July 2018

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The adult form of Niemann-Pick disease type C.

14 July 2018

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Role of cholesterol and lipid organization in disease

14 July 2018

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Elevated plasma membrane cholesterol content alters macrophage signaling and function.

14 July 2018

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Automated microscopy screening for compounds that partially revert cholesterol accumulation in Niemann-Pick C cells

14 July 2018

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Niemann-Pick type C disease and intracellular cholesterol trafficking

14 July 2018

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14 July 2018

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Therapy of Niemann-Pick disease, type C.

14 July 2018

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Lipid and cholesterol trafficking in NPC.

14 July 2018

1 reference

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Intracellular trafficking of Niemann-Pick C proteins 1 and 2: obligate components of subcellular lipid transport

14 July 2018

1 reference

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Structure and function of the NPC2 protein

14 July 2018

1 reference

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The NPC1 protein: structure implies function.

14 July 2018

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Cellular pathology of Niemann-Pick type C disease

14 July 2018

1 reference

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Niemann-Pick disease type C.

14 July 2018

1 reference

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Human acyl-coenzyme A:cholesterol acyltransferase expressed in chinese hamster ovary cells: membrane topology and active site location

14 July 2018

1 reference

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Endocytic trafficking of glycosphingolipids in sphingolipid storage diseases

14 July 2018

1 reference

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Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease

14 July 2018

1 reference

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Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes

14 July 2018

1 reference

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Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease

14 July 2018

1 reference

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Endocytosis and sorting of glycosphingolipids in sphingolipid storage disease

14 July 2018

1 reference

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Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells

14 July 2018

1 reference

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Phenotypic correction of lipid storage and growth arrest in wolman disease fibroblasts by gene transfer of lysosomal acid lipase

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Identification of HE1 as the second gene of Niemann-Pick C disease

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

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

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Intracellular cholesterol trafficking: role of the NPC1 protein

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-Golgi network

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

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

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Lipase activation by nonionic detergents. The crystal structure of the porcine lipase-colipase-tetraethylene glycol monooctyl ether complex

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Aurintricarboxylic acid, a putative inhibitor of apoptosis, is a potent inhibitor of DNA topoisomerase II in vitro and in Chinese hamster fibrosarcoma cells

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Wolman disease and its treatment

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Characterization of lysosomal acid lipase by site-directed mutagenesis and heterologous expression

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Aurintricarboxylic acid: inhibitor of initiation of protein synthesis

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Deficient activity of acid lipase in cholesterol-ester storage disease

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Esterification of low density lipoprotein cholesterol in human fibroblasts and its absence in homozygous familial hypercholesterolemia

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

A defect in cholesterol esterification in Niemann-Pick disease (type C) patients

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Functional expression of the human transferrin receptor cDNA in Chinese hamster ovary cells deficient in endogenous transferrin receptor

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Inhibition of pancreatic lipase in vitro by the covalent inhibitor tetrahydrolipstatin

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Identification of the active-site serine in human pancreatic lipase by chemical modification with tetrahydrolipstatin

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Inhibition of RNA-directed DNA polymerase by aurintricarboxylic acid

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Genetic variations and treatments that affect the lifespan of the NPC1 mouse

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Sterol, protein and lipid trafficking in Chinese hamster ovary cells with Niemann-Pick type C1 defect.

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

d-threo-1-Phenyl-2-decanoylamino-3-morpholino-1-propanol Alters Cellular Cholesterol Homeostasis by Modulating the Endosome Lipid Domains†

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Guilty until proven innocent: the case of NPC1 and cholesterol

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Protein transduction of Rab9 in Niemann-Pick C cells reduces cholesterol storage.

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Homogeneous fluorescent assay for RNA polymerase

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Hormone-sensitive lipase deficiency in mouse islets abolishes neutral cholesterol ester hydrolase activity but leaves lipolysis, acylglycerides, fat oxidation, and insulin secretion intact

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Differential trafficking of the Niemann-Pick C1 and 2 proteins highlights distinct roles in late endocytic lipid trafficking.

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Role of ABCA1 in cellular cholesterol efflux and reverse cholesterol transport

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Cyclodextrins in the treatment of a mouse model of Niemann-Pick C disease

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Enzyme therapy for lysosomal acid lipase deficiency in the mouse

25 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2783675

Effect of nonionic surfactants on Rhizopus homothallicus lipase activity: a comparative kinetic study

25 November 2018

1 reference

12 December 2020

Treatment perspectives for the lysosomal storage diseases

1 reference

12 December 2020

Acid lipase in cultured fibroblasts: cholesterol ester storage disease

1 reference

12 December 2020

Recognition and receptor-mediated endocytosis of the lysosomal acid lipase secreted by cultured human fibroblasts

1 reference

12 December 2020

Esterastin: a potent inhibitor of lysosomal acid lipase

1 reference

12 December 2020

Selective inhibition of acyl coenzyme A:cholesterol acyltransferase by compound 58-035

1 reference

12 December 2020

19 Receptor-mediated endocytosis of low-density lipoprotein in cultured cells

1 reference

12 December 2020

Reconstitution of the hydrophobic core of low-density lipoprotein

1 reference

12 December 2020

Studies on the antiobesity activity of tetrahydrolipstatin, a potent and selective inhibitor of pancreatic lipase

1 reference

12 December 2020

Mode of action of tetrahydrolipstatin: a derivative of the naturally occurring lipase inhibitor lipstatin

1 reference

12 December 2020

Localization of lipoprotein unesterified cholesterol in nondenaturing gradient gels with filipin

1 reference

12 December 2020

Effects of tetrahydrolipstatin, a lipase inhibitor, on absorption of fat from the intestine of the rat

1 reference

12 December 2020

Hormone-sensitive lipase is responsible for the neutral cholesterol ester hydrolase activity in macrophages

1 reference

12 December 2020

The lipase inhibitor tetrahydrolipstatin binds covalently to the putative active site serine of pancreatic lipase

1 reference

12 December 2020

Inactivation of pancreatic and gastric lipases by tetrahydrolipstatin and alkyl-dithio-5-(2-nitrobenzoic acid). A kinetic study with 1,2-didecanoyl-sn-glycerol monolayers

1 reference

12 December 2020

Absorption and fluorescence spectra of polyene antibiotics in the presence of cholesterol

1 reference

12 December 2020

Large spectral changes accompany the conformational transition of human pancreatic lipase induced by acylation with the inhibitor tetrahydrolipstatin

1 reference

12 December 2020

Intracellular trafficking of cholesterol monitored with a cyclodextrin

1 reference

12 December 2020

Suppression of neutral cholesterol ester hydrolase activity by antisense DNA of hormone-sensitive lipase

1 reference

12 December 2020

10.1016/J.BBALIP.2009.08.005

Identifiers

DOI

1 reference

20 August 2017

1 reference

20 August 2017

1 reference