(Q46665497)

24 December 2017

title

Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis (English)

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24 December 2017

author

Anneliese O Speak

2

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24 December 2017

Frances Platt

object named as

Frances M Platt

10

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author name string

Elena Elliot-Smith

1

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24 December 2017

Emyr Lloyd-Evans

3

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24 December 2017

David A Smith

4

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24 December 2017

Aarnoud C van der Spoel

5

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24 December 2017

Mylvaganam Jeyakumar

6

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24 December 2017

Terry D Butters

7

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24 December 2017

Raymond A Dwek

8

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24 December 2017

Alessandra d'Azzo

9

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24 December 2017

publication date

1 April 2008

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24 December 2017

Molecular Genetics and Metabolism

published in

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24 December 2017

volume

94

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24 December 2017

issue

2

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24 December 2017

page(s)

204-211

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24 December 2017

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

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1 reference

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Substrate reduction therapy for glycosphingolipid storage disorders

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

7 January 2021

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Generalized CNS disease and massive GM1-ganglioside accumulation in mice defective in lysosomal acid beta-galactosidase

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Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis

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

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

7 January 2021

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

7 January 2021

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N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis

7 January 2021

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

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

7 January 2021

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Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment

7 January 2021

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Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling

7 January 2021

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

7 January 2021

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

7 January 2021

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Abnormal transport along the lysosomal pathway in mucolipidosis, type IV disease

7 January 2021

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Brain gangliosides: functional ligands for myelin stability and the control of nerve regeneration

7 January 2021

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Ferret pyramidal cell dendritogenesis: changes in morphology and ganglioside expression during cortical development.

7 January 2021

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

7 January 2021

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The pharmacokinetics and tissue distribution of the glucosylceramide synthase inhibitor miglustat in the rat

7 January 2021

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

7 January 2021

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Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice

7 January 2021

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10.1016/J.YMGME.2008.02.005

7 January 2021

Identifiers

DOI

1 reference

24 December 2017

PubMed ID

18387328

1 reference

24 December 2017