Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types

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P1343

Q54836041-40ACAA5F-D3A9-410E-A2E5-96F9F8220986 Q54836504-34904782-400A-4A15-8C6C-09486AE3C85A Q54836506-85CD19AE-9D4C-4F37-9F4B-DDE5839E992D Q54837679-52091D40-2DB7-40AB-9F61-EA5A150AECA4 Q54837682-85C72FB5-29D2-481D-B00D-F1DF5B2C559C

P1343

Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types

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http://www.wikidata.org/entity/Q58794894

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im Oktober 2018 veröffentlichter wissenschaftlicher Artikel

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wetenschappelijk artikel

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наукова стаття, опублікована в жовтні 2018

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name

Glucosylceramide synthase inhi ...... with different mutation types

@en

Glucosylceramide synthase inhi ...... with different mutation types

@nl

type

Item

label

Glucosylceramide synthase inhi ...... with different mutation types

@en

Glucosylceramide synthase inhi ...... with different mutation types

@nl

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Glucosylceramide synthase inhi ...... with different mutation types

@en

Glucosylceramide synthase inhi ...... with different mutation types

@nl

P1476

Glucosylceramide synthase inhi ...... with different mutation types

@en

P2093

A Mühlemann

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M Garzotti

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M R Probst

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N Üçeyler

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O Morand

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P M A Groenen

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R W D Welford

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V Rickert

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P2860

Profile of eliglustat tartrate in the management of Gaucher disease

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A phase 1/2 clinical trial of enzyme replacement in fabry disease: pharmacokinetic, substrate clearance, and safety studies

Lucerastat, an iminosugar with potential as substrate reduction therapy for glycolipid storage disorders: safety, tolerability, and pharmacokinetics in healthy subjects

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease

Corrective effect on Fabry mice of yeast recombinant human alpha-galactosidase with N-linked sugar chains suitable for lysosomal delivery.

Enzyme replacement therapy with agalsidase alfa in patients with Fabry's disease: an analysis of registry data.

The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat

P304

3392-3403

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P31

scholarly article

P356

10.1093/HMG/DDY248

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P407

English

P433

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P478

http://www.w3.org/2001/XMLSchema#string

P577

2018-10-01T00:00:00Z

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29982630

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P921

molecular biology

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6140777

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Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types

about

P1343

P1343

Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types

description

name

type

label

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P1433

P1476

P2093

P2860

P304

P31

P356

P407

P433

P478

P577

P698

P921

P932

P356

P1433

P1476

P2093

P2860

P304

P31

P356

P407

P433

P478

P577

P698

P921

P932