Efficacy and ototoxicity of different cyclodextrins in Niemann-Pick C disease (Q28834418)

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3 April 2020

title

Efficacy and ototoxicity of different cyclodextrins in Niemann-Pick C disease (English)

1 reference

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Niemann–Pick disease, type C

3 April 2020

main subject

1 reference

1 reference

7

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3 April 2020

author name string

Cristin D Davidson

1

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3 April 2020

Yonatan I Fishman

2

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3 April 2020

István Puskás

3

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3 April 2020

Julianna Szemán

4

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3 April 2020

Tamás Sohajda

5

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3 April 2020

Leslie A McCauliff

6

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3 April 2020

Judith Storch

8

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3 April 2020

Marie T Vanier

9

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3 April 2020

Lajos Szente

10

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3 April 2020

Steven U Walkley

11

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3 April 2020

Kostantin Dobrenis

12

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3 April 2020

language of work or name

English

0 references

publication date

20 April 2016

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Annals of clinical and translational neurology

3 April 2020

published in

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volume

3

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3 April 2020

issue

5

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3 April 2020

page(s)

366-380

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

3 April 2020

cites work

1 reference

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Enhanced production of 24S-hydroxycholesterol is not sufficient to drive liver X receptor target genes in vivo

16 March 2017

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Cyclodextrin induces calcium-dependent lysosomal exocytosis

16 March 2017

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

16 March 2017

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Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression

16 March 2017

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

16 March 2017

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Different cellular traffic of LDL-cholesterol and acetylated LDL-cholesterol leads to distinct reverse cholesterol transport pathways

16 March 2017

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Mutagenesis of the putative sterol-sensing domain of yeast Niemann Pick C-related protein reveals a primordial role in subcellular sphingolipid distribution

16 March 2017

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Transmembrane molecular pump activity of Niemann-Pick C1 protein

16 March 2017

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Cholesterol homeostasis in human brain: turnover of 24S-hydroxycholesterol and evidence for a cerebral origin of most of this oxysterol in the circulation

16 March 2017

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Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activity

16 March 2017

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A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele

3 June 2018

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Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease

3 June 2018

1 reference

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HPGCD outperforms HPBCD as a potential treatment for Niemann-Pick disease type C during disease modeling with iPS cells.

3 June 2018

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Cyclodextrins, blood-brain barrier, and treatment of neurological diseases

3 June 2018

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Complex lipid trafficking in Niemann-Pick disease type C.

3 June 2018

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2-Hydroxypropyl-β-cyclodextrin promotes transcription factor EB-mediated activation of autophagy: implications for therapy

3 June 2018

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

3 June 2018

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Sterol transfer between cyclodextrin and membranes: similar but not identical mechanism to NPC2-mediated cholesterol transfer

3 June 2018

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Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.

3 June 2018

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

3 June 2018

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

3 June 2018

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

3 June 2018

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

3 June 2018

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Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells

3 June 2018

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Sparing methylation of beta-cyclodextrin mitigates cytotoxicity and permeability induction in respiratory epithelial cell layers in vitro

3 June 2018

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

3 June 2018

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

3 June 2018

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3 June 2018

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3 June 2018

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3 June 2018

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3 June 2018

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3 June 2018

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Consequences of cellular cholesterol accumulation: basic concepts and physiological implications

3 June 2018

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Probing red cell membrane cholesterol movement with cyclodextrin

3 June 2018

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Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations

3 June 2018

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3 June 2018

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3 June 2018

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Cellular cholesterol efflux mediated by cyclodextrins. Demonstration Of kinetic pools and mechanism of efflux.

3 June 2018

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3 June 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Influence of Npc1 genotype on the toxicity of hydroxypropyl-β-cyclodextrin, a potentially therapeutic agent, in Niemann-Pick Type C disease models.

21 January 2018

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Evaluation of the cytotoxicity of beta-cyclodextrin derivatives: evidence for the role of cholesterol extraction

21 January 2018

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Behavior of alpha-, beta-, and gamma-cyclodextrins and their derivatives on an in vitro model of blood-brain barrier.

21 January 2018

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Computational microscopy of cyclodextrin mediated cholesterol extraction from lipid model membranes

21 January 2018

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Kinetics of cholesterol extraction from lipid membranes by methyl-beta-cyclodextrin--a surface plasmon resonance approach

21 January 2018

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Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent

21 January 2018

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The autophagic defect in Niemann-Pick disease type C neurons differs from somatic cells and reduces neuronal viability.

21 January 2018

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γ-Cyclodextrin Increases Hydrolysis of Gangliosides by Sialidase from Arthrobacter ureafaciens: Hydrolysis of Gangliosides

21 January 2018

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Plasma levels of 27-hydroxycholesterol in humans and mice with monogenic disturbances of high density lipoprotein metabolism

21 January 2018

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12 December 2020

Efficacy of 2-Hydroxypropyl-β-cyclodextrin in Niemann-Pick Disease Type C Model Mice and Its Pharmacokinetic Analysis in a Patient with the Disease

1 reference

12 December 2020

Capillary electrophoresis

1 reference

12 December 2020

Preliminary safety evaluation of parenterally administered sulfoalkyl ether beta-cyclodextrin derivatives

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12 December 2020

Identifiers

DOI

10.1002/ACN3.306

2 references

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3 April 2020

1 reference

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3 April 2020

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