(Q80351466)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

title

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

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

1 reference

ChEBI release 2020-05-28

1 reference

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

John Marshall

12

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

author name string

Kerry Anne McEachern

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

John Fung

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Svetlana Komarnitsky

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Craig S Siegel

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Wei-Lien Chuang

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Elizabeth Hutto

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

James A Shayman

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Gregory A Grabowski

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Seng H Cheng

10

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

Diane P Copeland

11

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

publication date

16 May 2007

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

Molecular Genetics and Metabolism

30 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

volume

91

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

issue

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

page(s)

259-267

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

Lysosomal storage disorders

30 December 2019

cites work

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Sphingolipid metabolism diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Gaucher disease: Perspectives on a prototype lysosomal disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Limitations of enzyme replacement therapy: current and future

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Remaining problems in the management of patients with Gaucher disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

New prospects for the treatment of lysosomal storage diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Treatment of Gaucher disease with an enzyme inhibitor

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Substrate reduction therapy for glycosphingolipid storage disorders

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Biochemistry of glycosphingolipid storage disorders: implications for therapeutic intervention

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Substrate reduction therapy of glycosphingolipid storage disorders.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Low-dose N-butyldeoxynojirimycin (OGT 918) for type I Gaucher disease.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

An open-label, noncomparative study of miglustat in type I Gaucher disease: efficacy and tolerability over 24 months of treatment

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Studies of the action of ceramide-like substances (D- and L-PDMP) on sphingolipid glycosyltransferases and purified lactosylceramide synthase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Improved inhibitors of glucosylceramide synthase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Improved Inhibitors of Glucosylceramide Synthase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Glycosphingolipid depletion in fabry disease lymphoblasts with potent inhibitors of glucosylceramide synthase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Mouse models of human lysosomal diseases.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Substrate reduction therapy in mouse models of the glycosphingolipidoses

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Inhibition of glycogen breakdown by imino sugars in vitro and in vivo.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Inhibiting glycosphingolipid synthesis improves glycemic control and insulin sensitivity in animal models of type 2 diabetes

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Analysis of the lipids of normal and Gaucher bone marrow.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

AAV8-mediated expression of glucocerebrosidase ameliorates the storage pathology in the visceral organs of a mouse model of Gaucher disease.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Sphingolipid activator proteins are required for epidermal permeability barrier formation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Replacement therapy for inherited enzyme deficiency--macrophage-targeted glucocerebrosidase for Gaucher's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Gaucher disease: lessons from a decade of therapy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Glucocerebrosidase level in the cerebrospinal fluid during enzyme replacement therapy--unsuccessful treatment of the neurological abnormality in type 2 Gaucher disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Beneficial effect of pre-transplant splenectomy on displacement bone marrow transplantation for Gaucher's syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

Sustained therapeutic effects of oral miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) in type I Gaucher disease.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2007.04.001

10.1016/J.YMGME.2007.04.001

7 January 2021

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:17509920%20AND%20SRC:MED&resulttype=core&format=json

30 December 2019

1 reference