Unraveling the sterol-trafficking defect in Niemann-Pick C disease (Q37114726)

18 August 2017

Unraveling the sterol-trafficking defect in Niemann-Pick C disease (English)

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Niemann–Pick disease, type C

18 August 2017

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Stephen L Sturley

1

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18 August 2017

Marc C Patterson

series ordinal

2

1 reference

18 August 2017

Peter Pentchev

series ordinal

3

1 reference

18 August 2017

language of work or name

English

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publication date

11 February 2009

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Proceedings of the National Academy of Sciences of the United States of America

18 August 2017

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18 August 2017

106

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18 August 2017

7

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18 August 2017

2093-2094

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

18 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

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

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein.

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2650112

Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation

3 September 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/19211789

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/19211789

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1073/PNAS.0812934106

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18 August 2017

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18 August 2017

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