(Q45248629)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15687347%20AND%20SRC:MED&resulttype=core&format=json

8 January 2020

title

Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice (English)

1 reference

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8 January 2020

5

1 reference

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8 January 2020

Frances Platt

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15687347%20AND%20SRC:MED&resulttype=core&format=json

8 January 2020

author name string

Kasperzyk JL

1

1 reference

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8 January 2020

d'Azzo A

2

1 reference

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8 January 2020

Alroy J

4

1 reference

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8 January 2020

language of work or name

1 reference

1 February 2005

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15687347%20AND%20SRC:MED&resulttype=core&format=json

Journal of Lipid Research

8 January 2020

published in

1 reference

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8 January 2020

volume

46

1 reference

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8 January 2020

page(s)

744-751

1 reference

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8 January 2020

issue

4

1 reference

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Expression of human beta-hexosaminidase alpha-subunit gene (the gene defect of Tay-Sachs disease) in mouse brains upon engraftment of transduced progenitor cells

8 January 2020

cites work

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

21 January 2018

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Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis

21 January 2018

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New prospects for the treatment of lysosomal storage diseases

21 January 2018

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Attenuation of ganglioside GM1 accumulation in the brain of GM1 gangliosidosis mice by neonatal intravenous gene transfer

21 January 2018

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Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients.

21 January 2018

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Enzyme replacement therapy: conception, chaos and culmination

21 January 2018

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Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases

21 January 2018

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Small-molecule therapeutics for the treatment of glycolipid lysosomal storage disorders

21 January 2018

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Expression of glucosylceramide synthase, converting ceramide to glucosylceramide, confers adriamycin resistance in human breast cancer cells

21 January 2018

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

21 January 2018

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Therapeutic applications of imino sugars in lysosomal storage disorders

21 January 2018

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Substrate reduction therapy in mouse models of the glycosphingolipidoses

21 January 2018

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

21 January 2018

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The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement

21 January 2018

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Workshop W2: New Insights on Inherited Neurological Storage Diseases

21 January 2018

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21 January 2018

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Cellular localization of gangliosides in the developing mouse cerebellum: analysis using the weaver mutant.

21 January 2018

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Heterosis for brain myelin content in mice.

21 January 2018

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Biochemical changes in mouse brain composition during myelination.

21 January 2018

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Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA/2J and knockout mice.

21 January 2018

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Cerebral, cerebellar, and brain stem gangliosides in mice susceptible to audiogenic seizures.

21 January 2018

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A SIMPLE AND ACCURATE MICROMETHOD FOR QUANTITATIVE DETERMINATION OF GANGLIOSIDE PATTERNS.

21 January 2018

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High-performance thin-layer chromatography and densitometric determination of brain ganglioside compositions of several species

21 January 2018

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Estimation of monosaccharides by the orcinol–sulphuric acid reaction

21 January 2018

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Genetic analysis of cerebellar lipids in mice susceptible to audiogenic seizures.

21 January 2018

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Cellular distribution of gangliosides in the developing mouse cerebellum: analysis using the staggerer mutant.

21 January 2018

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Ganglioside abnormalities associated with failed neural differentiation in a T-locus mutant mouse embryo.

21 January 2018

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Phylogenetic conservation of ganglioside GD3 expression during early vertebrate ontogeny

21 January 2018

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Changes of ganglioside pattern during cerebellar development of normal and staggerer mice

21 January 2018

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GD3 ganglioside is a glycolipid characteristic of immature neuroectodermal cells.

21 January 2018

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A reappraisal of ganglioside GD3 expression in the CNS.

21 January 2018

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Gangliosides in developing mouse brain myelin

21 January 2018

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Sialosylgalactosyl ceramide as a specific marker for human myelin and oligodendroglial perikarya: gangliosides of human myelin, oligodendroglia and neurons

21 January 2018

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Development of lysosomal storage in mice with targeted disruption of the beta-galactosidase gene: a model of human G(M1)-gangliosidosis.

21 January 2018

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

21 January 2018

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