(Q34812413)

4 August 2017

title

Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease (English)

1 reference

4 August 2017

Blair R Roszell

1

1 reference

4 August 2017

Jian-Qin Tao

2

1 reference

4 August 2017

Kevin J Yu

3

1 reference

4 August 2017

Ling Gao

4

1 reference

4 August 2017

Shaohui Huang

5

1 reference

4 August 2017

Yue Ning

6

1 reference

4 August 2017

Sheldon I Feinstein

7

1 reference

4 August 2017

Charles H Vite

8

1 reference

4 August 2017

Sandra R Bates

9

1 reference

4 August 2017

language of work or name

English

0 references

publication date

2 July 2013

1 reference

4 August 2017

1 reference

4 August 2017

volume

8

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4 August 2017

issue

7

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4 August 2017

page(s)

e67084

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Creative Commons Attribution 4.0 International

4 August 2017

copyright license

start time

2 July 2013

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

describes a project that uses

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3699545/fullTextXML

inferred from PubMed Central ID database lookup

cites work

Characterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lung

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Free cholesterol accumulation impairs antioxidant activities and aggravates apoptotic cell death in menadione-induced oxidative injury

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1⁻/⁻ mice

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

A role for oxysterol-binding protein-related protein 5 in endosomal cholesterol trafficking

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Niemann‐Pick type C disease: molecular mechanisms and potential therapeutic approaches

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Niemann-Pick disease type C

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Conditional deletion of Abca3 in alveolar type II cells alters surfactant homeostasis in newborn and adult mice.

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

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

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1-/- mice

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

An elevated level of cholesterol impairs self-assembly of pulmonary surfactant into a functional film

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

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

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Protein measurement with the Folin phenol reagent

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

A RAPID METHOD OF TOTAL LIPID EXTRACTION AND PURIFICATION

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Phosphorus assay in column chromatography

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Identification and characterization of rodent ABCA1 in isolated type II pneumocytes

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactant

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Niemann-Pick disease type C: spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Clinical-biochemical correlation in molecularly characterized patients with Niemann-Pick type C.

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

The roles of cholesterol in pulmonary surfactant: insights from comparative and evolutionary studies

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

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

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

The role of lipids in pulmonary surfactant.

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Murine Model of Niemann-Pick C Disease: Mutation in a Cholesterol Homeostasis Gene

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

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

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Feline Niemann-Pick disease type C

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Surfactant cholesterol metabolism of the isolated perfused rat lung

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Mutation analysis of feline Niemann-Pick C1 disease

8 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0067084

Pulmonary surfactant function is abolished by an elevated proportion of cholesterol

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0067084

Role of cholesterol in the biophysical dysfunction of surfactant in ventilator-induced lung injury.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0067084

SP-A is necessary for increased clearance of alveolar DPPC with hyperventilation or secretagogues

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0067084

Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling/degeneration (giant lamellar body degeneration) of type-2 pneumocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0067084

Macrophage and type II cell catabolism of SP-A and saturated phosphatidylcholine in mouse lungs

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3699545

Surface active materials from dog lung. II. Composition and physiological correlations

26 October 2018

1 reference

12 December 2020

Isolation of disaturated phosphatidylcholine with osmium tetroxide

1 reference

12 December 2020

Lung lamellar bodies maintain an acidic internal pH

1 reference

12 December 2020

Ultrastructural changes in the lung in Niemann-Pick type C mouse

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0067084

Identifiers

DOI

1 reference

4 August 2017

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4 August 2017

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