(Q37209173)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

title

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

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

0 references

1 reference

5

1 reference

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8 December 2019

Frances Platt

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

author name string

M Jeyakumar

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

T D Butters

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

M Cortina-Borja

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

V Hunnam

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

V H Perry

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

R A Dwek

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

language of work or name

English

0 references

publication date

1 May 1999

1 reference

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8 December 2019

number of pages

6

1 reference

Proceedings of the National Academy of Sciences of the United States of America

inferred from page(s)

published in

1 reference

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8 December 2019

volume

96

1 reference

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8 December 2019

issue

11

1 reference

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8 December 2019

page(s)

6388-6393

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

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

8 December 2019

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

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

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

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

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

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

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Gene therapy of lysosomal storage disorders

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Microglia: The Effector Cell for Reconstitution of the Central Nervous System following Bone Marrow Transplantation for Lysosomal and Peroxisomal Storage Diseases

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Treatment of Gaucher disease with an enzyme inhibitor

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Ganglioside metabolism--topology and regulation

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

New therapeutic prospects for the glycosphingolipid lysosomal storage diseases.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=26891

Allogeneic bone marrow transplantation for lysosomal storage diseases. The European Group for Bone Marrow Transplantation

3 September 2018

1 reference

12 December 2020

Extensive Glycosphingolipid Depletion in the Liver and Lymphoid Organs of Mice Treated withN-Butyldeoxynojirimycin

1 reference

12 December 2020

The string test: an early behavioral change in thiamine deficiency

1 reference

12 December 2020

Enzyme replacement therapy for Gaucher disease

1 reference

12 December 2020

Enzyme therapy in type 1 Gaucher disease: comparative efficacy of mannose-terminated glucocerebrosidase from natural and recombinant sources

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.96.11.6388

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:10339597%20AND%20SRC:MED&resulttype=core&format=json

8 December 2019

1 reference