Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease (Q28478879)

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

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scholarly article

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

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Sandhoff disease

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object named as

John Marshall

13

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author name string

Karen M Ashe

1

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Dinesh Bangari

2

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Lingyun Li

3

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Mario A Cabrera-Salazar

4

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Scott D Bercury

5

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Jennifer B Nietupski

6

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Christopher G F Cooper

7

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Johannes M F G Aerts

8

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Edward R Lee

9

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Diane P Copeland

10

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Seng H Cheng

11

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Ronald K Scheule

12

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publication date

2011

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6

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6

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e21758

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Creative Commons Attribution 4.0 International

29 June 2011

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April 2022 Public Data File from Crossref

copyright status

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Pharmacological inhibition of glucosylceramide synthase enhances insulin sensitivity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

19 March 2017

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

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

19 March 2017

NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin

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19 March 2017

Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice

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19 March 2017

Long-term safety and efficacy of enzyme replacement therapy for Fabry disease

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Neuronal accumulation of alpha- and beta-synucleins in the brain of a GM2 gangliosidosis mouse model

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19 March 2017

A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder

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19 March 2017

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease

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19 March 2017

Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin

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19 March 2017

Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases

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19 March 2017

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism

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19 March 2017

Alpha-synuclein activates microglia by inducing the expressions of matrix metalloproteinases and the subsequent activation of protease-activated receptor-1.

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29 September 2017

A phase 2 study of eliglustat tartrate (Genz-112638), an oral substrate reduction therapy for Gaucher disease type 1.

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29 September 2017

Pathophysiology of neuropathic lysosomal storage disorders

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Therapeutic approaches for neuronopathic lysosomal storage disorders

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29 September 2017

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

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

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Substrate reduction therapy in juvenile GM2 gangliosidosis

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

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29 September 2017

Randomized, controlled trial of miglustat in Gaucher's disease type 3.

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29 September 2017

Sphingosine kinase 1/S1P receptor signaling axis controls glial proliferation in mice with Sandhoff disease.

1 reference

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29 September 2017

Outcome of type III Gaucher disease on enzyme replacement therapy: review of 55 cases.

1 reference

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29 September 2017

Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.

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29 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Sphingolipidomics: high-throughput, structure-specific, and quantitative analysis of sphingolipids by liquid chromatography tandem mass spectrometry

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Substrate reduction therapy in mouse models of the glycosphingolipidoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Effectiveness of enzyme replacement therapy in 1028 patients with type 1 Gaucher disease after 2 to 5 years of treatment: a report from the Gaucher Registry

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3126858

29 September 2017

Substrate reduction therapy for glycosphingolipid storage disorders

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29 September 2017

Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation

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29 September 2017

Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.

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29 September 2017

Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice

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29 September 2017

Recovery of motor function after spinal-cord injury--a randomized, placebo-controlled trial with GM-1 ganglioside

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2 June 2018

Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis.

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21 January 2018

Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice.

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21 January 2018

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease.

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https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0021758

21 January 2018

Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.

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https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0021758

21 January 2018

Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis

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21 January 2018

Inhibition of glycogen biosynthesis via mTORC1 suppression as an adjunct therapy for Pompe disease.

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21 January 2018

Miglustat in late-onset Tay-Sachs disease: a 12-month, randomized, controlled clinical study with 24 months of extended treatment

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https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0021758

21 January 2018

Molecular pathophysiology in Tay-Sachs and Sandhoff diseases as revealed by gene expression profiling.

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https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0021758

21 January 2018

Inhibition of glycolipid biosynthesis by N-(5-adamantane-1-yl-methoxy-pentyl)-deoxynojirimycin protects against the inflammatory response in hapten-induced colitis.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0021758

21 January 2018

Aggregated alpha-synuclein activates microglia: a process leading to disease progression in Parkinson's disease

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21 January 2018

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

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27 November 2018

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

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12 December 2020

based on heuristic

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21738789

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

No justification for very high-dose enzyme therapy for patients with type III Gaucher disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21738789

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Substrate reduction therapy with miglustat in chronic GM2 gangliosidosis type Sandhoff: results of a 3-year follow-up

1 reference

https://pubmed.ncbi.nlm.nih.gov/21738789

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1371/JOURNAL.PONE.0021758

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Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

2011PLoSO...621758A

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Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

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Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

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Consolidated OpenCitations Corpus – April 2017

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ResearchGate publication ID

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