(Q48641010)

English

Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann-Pick C mice.

scientific article published in February 2012

In more languages

Statements

scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann-Pick C mice (English)

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Niemann–Pick disease, type C

1 reference

based on heuristic

inferred from title

9

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

13

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

author name string

Jennifer B Nietupski

1

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Joshua J Pacheco

2

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Wei-Lien Chuang

3

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Kimberly Maratea

4

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Lingyun Li

5

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Joseph Foley

6

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Karen M Ashe

7

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Christopher G F Cooper

8

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Diane P Copeland

10

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Ronald K Scheule

11

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Seng H Cheng

12

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

publication date

1 February 2012

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Molecular Genetics and Metabolism

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

105

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

4

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

621-628

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Secondary accumulation of gangliosides in lysosomal storage disorders

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Pharmacological and genetic modifications of somatic cholesterol do not substantially alter the course of CNS disease in Niemann-Pick C mice.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

24 month-treatment with miglustat of three patients with Niemann-Pick disease type C: follow up using brain spectroscopy

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Miglustat in patients with Niemann-Pick disease Type C (NP-C): a multicenter observational retrospective cohort study

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Miglustat in adult and juvenile patients with Niemann-Pick disease type C: long-term data from a clinical trial

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Critical role for glycosphingolipids in Niemann-Pick disease type C.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Generation of specific deoxynojirimycin-type inhibitors of the non-lysosomal glucosylceramidase

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Dual-action lipophilic iminosugar improves glycemic control in obese rodents by reduction of visceral glycosphingolipids and buffering of carbohydrate assimilation

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Intracerebroventricular delivery of glucocerebrosidase reduces substrates and increases lifespan in a mouse model of neuronopathic Gaucher disease.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Replacement of chloroform throughout glycosphingolipid isolation

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Niemann-Pick type C pathogenesis and treatment: from statins to sugars.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Pharmacological small molecules for the treatment of lysosomal storage disorders.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Rescue of neurodegeneration in Niemann-Pick C mice by a prion-promoter-driven Npc1 cDNA transgene.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Decreased estradiol release from astrocytes contributes to the neurodegeneration in a mouse model of Niemann-Pick disease type C

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Reducing glycosphingolipid content in adipose tissue of obese mice restores insulin sensitivity, adipogenesis and reduces inflammation

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Accumulation of Glucosylceramide in Murine Testis, Caused by Inhibition of β-Glucosidase 2

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Identification of the non-lysosomal glucosylceramidase as beta-glucosidase 2

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Mutation of beta-glucosidase 2 causes glycolipid storage disease and impaired male fertility

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Biochemistry of glycosphingolipid storage disorders: implications for therapeutic intervention

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.01.020

7 January 2021

Identifiers

10.1016/J.YMGME.2012.01.020

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22366055%20AND%20SRC:MED&resulttype=core&format=json

24 January 2020

Sitelinks

Wikipedia(0 entries)

Wikibooks(0 entries)

Wikinews(0 entries)

Wikiquote(0 entries)

Wikisource(0 entries)

Wikiversity(0 entries)

Wikivoyage(0 entries)

Wiktionary(0 entries)

Multilingual sites(0 entries)

Retrieved from "https://www.wikidata.org/w/index.php?title=Q48641010&oldid=1424622556"