(Q33886270)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

title

Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

1 reference

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

10

Yusuf A Hannun

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

author name string

Ying Sun

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Huimin Ran

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Benjamin Liou

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Brian Quinn

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Matt Zamzow

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Wujuan Zhang

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Jacek Bielawski

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Kenneth D R Setchell

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

Gregory A Grabowski

11

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

language of work or name

English

0 references

publication date

20 April 2011

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

volume

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

issue

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

page(s)

e19037

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

Creative Commons Attribution 4.0 International

30 January 2020

copyright license

start time

20 April 2011

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

describes a project that uses

ImageQuant

object named as

ImageQuant

2 references

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3080394/fullTextXML

inferred from PubMed Central ID database lookup

CZ Software Mentions: A large dataset of software mentions in the biomedical literature

cites work

Type 2 Gaucher disease: phenotypic variation and genotypic heterogeneity

1 reference

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Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model

5 July 2018

1 reference

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

5 July 2018

1 reference

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Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Effects of pH and Iminosugar Pharmacological Chaperones on Lysosomal Glycosidase Structure and Stability

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Involvement of acid beta-glucosidase 1 in the salvage pathway of ceramide formation

5 July 2018

1 reference

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Acid beta-glucosidase 1 counteracts p38delta-dependent induction of interleukin-6: possible role for ceramide as an anti-inflammatory lipid

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Dose-response relationships for enzyme replacement therapy with imiglucerase/alglucerase in patients with Gaucher disease type 1.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

A novel p38 alpha MAPK inhibitor suppresses brain proinflammatory cytokine up-regulation and attenuates synaptic dysfunction and behavioral deficits in an Alzheimer's disease mouse model.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Novel strategies for inhibition of the p38 MAPK pathway.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Alpha-synuclein activates stress signaling protein kinases in THP-1 cells and microglia

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3080394

Guidance on the use of miglustat for treating patients with type 1 Gaucher disease

5 July 2018

1 reference

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Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses

5 July 2018

1 reference

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

5 July 2018

1 reference

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Acidic mammalian chitinase in asthmatic Th2 inflammation and IL-13 pathway activation

5 July 2018

1 reference

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Viable mouse models of acid beta-glucosidase deficiency: the defect in Gaucher disease

5 July 2018

1 reference

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Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease

5 July 2018

1 reference

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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

5 July 2018

1 reference

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Rationales for cancer chemotherapy with PDMP, a specific inhibitor of glucosylceramide synthase

5 July 2018

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Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation

5 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0019037

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0019037

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

21 January 2018

1 reference

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Gem-diamine 1-N-iminosugars as versatile glycomimetics: synthesis, biological activity and therapeutic potential.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0019037

Gaucher disease mouse models: point mutations at the acid beta-glucosidase locus combined with low-level prosaposin expression lead to disease variants.

21 January 2018

1 reference

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Turnover and distribution of intravenously administered mannose-terminated human acid beta-glucosidase in murine and human tissues.

21 January 2018

1 reference

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A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21533102

12 December 2020

inferred from PubMed ID database lookup

Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21533102

12 December 2020

inferred from PubMed ID database lookup

Effect of enzyme replacement therapy with imiglucerase on BMD in type 1 Gaucher disease

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21533102

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1371/JOURNAL.PONE.0019037

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21533102%20AND%20SRC:MED&resulttype=core&format=json

30 January 2020

1 reference