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Metabolic Control of Astrocyte Pathogenic Activity via cPLA2-MAVS

Cell, 2019-12, Vol.179 (7), p.1483-1498.e22

2019

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Miglustat in Niemann-Pick disease type C patients: a review

Orphanet journal of rare diseases, 2018-08, Vol.13 (1), p.140-140, Article 140

2018

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Case report of the perinatal form of Nieman-Pick disease type С against the backdrop of pathogenetic off-label therapy

Pediatricheskai͡a︡ farmakologii͡a︡ : nauchno-prakticheskiĭ zhurnal Soi͡u︡za pediatrov Rossii, 2024-05, Vol.21 (2), p.126-130

2024

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Long‐term survival outcomes of patients with Niemann‐Pick disease type C receiving miglustat treatment: A large retrospective observational study

Journal of inherited metabolic disease, 2020-09, Vol.43 (5), p.1060-1069

2020

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Sphingolipid lysosomal storage disorders

Nature (London), 2014-06, Vol.510 (7503), p.68-75

Platt, Frances M.

2014

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Iminosugar Glucosidase Inhibitors Reduce Hepatic Inflammation in Hepatitis A Virus-Infected Ifnar1 -/- Mice

Journal of virology, 2021-05, Vol.95 (11), p.e0005821-e0005821

2021

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Twelve years of experience with miglustat in the treatment of type 1 Gaucher disease: The Spanish ZAGAL project

Blood cells, molecules, & diseases, 2018-02, Vol.68, p.173-179

2018

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Current advancements in therapy for Niemann-Pick disease: progress and pitfalls

Expert opinion on pharmacotherapy, 2023-07, Vol.24 (11), p.1229-1247

2023

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Different Trafficking Phenotypes of Niemann-Pick C1 Gene Mutations Correlate with Various Alterations in Lipid Storage, Membrane Composition and Miglustat Amenability

International journal of molecular sciences, 2020-03, Vol.21 (6), p.2101

2020

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Differently increased volumes of multiple brain areas in Npc1 mutant mice following various drug treatments

Frontiers in neuroanatomy, 2024-07, Vol.18, p.1430790

2024

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Exploiting Gangliosides for the Therapy of Ewing's Sarcoma and H3K27M-Mutant Diffuse Midline Glioma

Cancers, 2021-01, Vol.13 (3), p.520

2021

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Cytokine profile and cholesterol levels in patients with Niemann-Pick type C disease presenting neurological symptoms: in vivo effect of miglustat and in vitro effect of N-acetylcysteine and coenzyme Q10

Experimental cell research, 2022-07, Vol.416 (2), p.113175-113175, Article 113175

2022

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Miglustat, a glucosylceramide synthase inhibitor, mitigates liver fibrosis through TGF-β/Smad pathway suppression in hepatic stellate cells

Biochemical and biophysical research communications, 2023-01, Vol.642, p.192-200

2023

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Treatment outcomes following continuous miglustat therapy in patients with Niemann-Pick disease Type C: a final report of the NPC Registry

Orphanet journal of rare diseases, 2020-04, Vol.15 (1), p.104-104, Article 104

2020

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Efficacy and safety of miglustat in the treatment of GM2 gangliosidosis: A systematic review

European journal of neurology, 2023-09, Vol.30 (9), p.2919-2945

2023

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Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders

International Journal of Molecular Sciences, 2016-07, Vol.17 (7), p.1065-1065

2016

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Profil des troubles cognitifs dans la maladie de Niemann Pick de type C

Revue neurologique, 2017-03, Vol.173, p.S141-S141

2017

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Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches

Journal of neurochemistry, 2011-03, Vol.116 (5), p.789-795

2011

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Infantile gangliosidoses: Mapping a timeline of clinical changes

Molecular genetics and metabolism, 2017-06, Vol.121 (2), p.170-179

2017

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Inhibitors of Protein Glycosylation Are Active against the Coronavirus Severe Acute Respiratory Syndrome Coronavirus SARS-CoV-2

Viruses, 2021-04, Vol.13 (5), p.808

2021

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Iminosugars: Effects of Stereochemistry, Ring Size, and N -Substituents on Glucosidase Activities

Pharmaceuticals (Basel, Switzerland), 2019-07, Vol.12 (3), p.108

2019

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Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann-Pick-Disease Type C1

International journal of molecular sciences, 2018-03, Vol.19 (4), p.972

2018

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Elevated cerebrospinal fluid ubiquitin C-terminal hydrolase-L1 levels correlate with phenotypic severity and therapeutic response in Niemann-Pick disease, type C1

Molecular genetics and metabolism, 2023-11, Vol.140 (3), p.107656-107656, Article 107656

2023

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FDA orphan drug designations for lysosomal storage disorders - a cross-sectional analysis

PloS one, 2020-04, Vol.15 (4), p.e0230898-e0230898

2020

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Acetyl-leucine slows disease progression in lysosomal storage disorders

Brain communications, 2021-01, Vol.3 (1), p.fcaa148-fcaa148

2021

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Metabolic Control of Astrocyte Pathogenic Activity via cPLA2-MAVS

Miglustat in Niemann-Pick disease type C patients: a review

Case report of the perinatal form of Nieman-Pick disease type С against the backdrop of pathogenetic off-label therapy

Long‐term survival outcomes of patients with Niemann‐Pick disease type C receiving miglustat treatment: A large retrospective observational study

Sphingolipid lysosomal storage disorders

Iminosugar Glucosidase Inhibitors Reduce Hepatic Inflammation in Hepatitis A Virus-Infected Ifnar1 -/- Mice

Twelve years of experience with miglustat in the treatment of type 1 Gaucher disease: The Spanish ZAGAL project

Current advancements in therapy for Niemann-Pick disease: progress and pitfalls

Different Trafficking Phenotypes of Niemann-Pick C1 Gene Mutations Correlate with Various Alterations in Lipid Storage, Membrane Composition and Miglustat Amenability

Differently increased volumes of multiple brain areas in Npc1 mutant mice following various drug treatments

Exploiting Gangliosides for the Therapy of Ewing's Sarcoma and H3K27M-Mutant Diffuse Midline Glioma

Cytokine profile and cholesterol levels in patients with Niemann-Pick type C disease presenting neurological symptoms: in vivo effect of miglustat and in vitro effect of N-acetylcysteine and coenzyme Q10

Miglustat, a glucosylceramide synthase inhibitor, mitigates liver fibrosis through TGF-β/Smad pathway suppression in hepatic stellate cells

Treatment outcomes following continuous miglustat therapy in patients with Niemann-Pick disease Type C: a final report of the NPC Registry

Efficacy and safety of miglustat in the treatment of GM2 gangliosidosis: A systematic review

Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders

Profil des troubles cognitifs dans la maladie de Niemann Pick de type C

Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches

Infantile gangliosidoses: Mapping a timeline of clinical changes

Inhibitors of Protein Glycosylation Are Active against the Coronavirus Severe Acute Respiratory Syndrome Coronavirus SARS-CoV-2

Iminosugars: Effects of Stereochemistry, Ring Size, and N -Substituents on Glucosidase Activities

Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann-Pick-Disease Type C1

Elevated cerebrospinal fluid ubiquitin C-terminal hydrolase-L1 levels correlate with phenotypic severity and therapeutic response in Niemann-Pick disease, type C1

FDA orphan drug designations for lysosomal storage disorders - a cross-sectional analysis

Acetyl-leucine slows disease progression in lysosomal storage disorders

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