(Q35920269)

English

Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function

scientific article published in October 2014

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function (English)

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

author name string

Adam M Lopez

1

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Sandi J Terpack

2

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Kenneth S Posey

3

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Benny Liu

4

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Charina M Ramirez

5

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Stephen D Turley

6

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

publication date

1 October 2014

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Clinical and Experimental Pharmacology and Physiology

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

41

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

10

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

780-787

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Lysosomal-specific cholesterol reduction by biocleavable polyrotaxanes for ameliorating Niemann-Pick type C disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Synthesis, characterization, and evaluation of pluronic-based β-cyclodextrin polyrotaxanes for mobilization of accumulated cholesterol from Niemann-Pick type C fibroblasts

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Clinical effect and safety profile of recombinant human lysosomal acid lipase in patients with cholesteryl ester storage disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Hearing loss and hair cell death in mice given the cholesterol-chelating agent hydroxypropyl-β-cyclodextrin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Effects of cyclodextrin in two patients with Niemann-Pick Type C disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Neuroprotective efficacy of aminopropyl carbazoles in a mouse model of amyotrophic lateral sclerosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Protein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Amino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Niemann-Pick disease type C

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Miglustat for treatment of Niemann-Pick C disease: a randomised controlled study

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

2-Hydroxypropyl-beta-cyclodextrin (HP-beta-CD): a toxicology review

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Pharmaceutical applications of cyclodextrins. III. Toxicological issues and safety evaluation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

11 July 2018

Reversal of advanced disease in lysosomal acid lipase deficient mice: a model for lysosomal acid lipase deficiency disease

1 reference

https://api.crossref.org/works/10.1111%2F1440-1681.12285

21 January 2018

Uptake of a fluorescent methyl-β-cyclodextrin via clathrin-dependent endocytosis.

1 reference

https://api.crossref.org/works/10.1111%2F1440-1681.12285

21 January 2018

Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4526211

24 September 2018

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/25115571

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lung toxicity of hydroxypropyl-β-cyclodextrin infusion

1 reference

https://pubmed.ncbi.nlm.nih.gov/25115571

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lysosomal acid lipase deficiency in rats: lipid analyses and lipase activities in liver and spleen

1 reference

https://pubmed.ncbi.nlm.nih.gov/25115571

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Marked reduction in bile acid synthesis in cholesterol 7alpha-hydroxylase-deficient mice does not lead to diminished tissue cholesterol turnover or to hypercholesterolemia

1 reference

https://pubmed.ncbi.nlm.nih.gov/25115571

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1111/1440-1681.12285

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

11 August 2017

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