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NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress

Nature communications, 2019-09, Vol.10 (1), p.4276-14, Article 4276

2019

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A master protocol to investigate a novel therapy acetyl-L-leucine for three ultra-rare neurodegenerative diseases: Niemann-Pick type C, the GM2 gangliosidoses, and ataxia telangiectasia

Trials, 2021-01, Vol.22 (1), p.84-84, Article 84

2021

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Terror and Greatness: Ivan and Peter as Russian Myths

Platt, Kevin M. F

1, 2011

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

Journal of inherited metabolic disease, 2004-11, Vol.27 (6), p.757-766

2004

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

Brain (London, England : 1878), 2003-04, Vol.126 (4), p.974-987

2003

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Inactivation of the Rb pathway and overexpression of both isoforms of E2F3 are obligate events in bladder tumours with 6p22 amplification

Oncogene, 2008-04, Vol.27 (19), p.2716-2727

2008

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Putative tumour suppressor gene necdin is hypermethylated and mutated in human cancer

British journal of cancer, 2013-04, Vol.108 (6), p.1368-1377

2013

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Autophagy Induction and Autophagosome Clearance in Neurons: Relationship to Autophagic Pathology in Alzheimer's Disease

The Journal of neuroscience, 2008-07, Vol.28 (27), p.6926-6937

2008

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

Journal of lipid research, 2005-04, Vol.46 (4), p.744-751

2005

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A Conversation with Maria Stepanova

World literature today, 2023-03, Vol.97 (2), p.42-45

2023

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Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis

Neuropathology and applied neurobiology, 2002-10, Vol.28 (5), p.343-357

2002

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N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis

The Journal of biological chemistry, 1994-03, Vol.269 (11), p.8362-8365

1994

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

Journal of inherited metabolic disease, 2007-02, Vol.30 (1), p.79-87

2007

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Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy

Journal of inherited metabolic disease, 2001-04, Vol.24 (2), p.275-290

2001

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Prevention of Lysosomal Storage in Tay-Sachs Mice Treated with N-Butyldeoxynojirimycin

Science (American Association for the Advancement of Science), 1997-04, Vol.276 (5311), p.428-431

1997

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In-depth phenotyping for clinical stratification of Gaucher disease

Orphanet journal of rare diseases, 2021-10, Vol.16 (1), p.431-431, Article 431

2021

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

Biochemical pharmacology, 2000-04, Vol.59 (7), p.821-829

2000

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N-butyldeoxynojirimycin causes weight loss as a result of appetite suppression in lean and obese mice

Diabetes, obesity & metabolism, 2008-02, Vol.10 (2), p.159-166

2008

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New therapeutic prospects for the glycosphingolipid lysosomal storage diseases

Biochemical pharmacology, 1998-08, Vol.56 (4), p.421-430

1998

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The Russophone Literature of Resistance: Introduction

World literature today, 2023-03, Vol.97 (2), p.38-41

2023

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The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding

Journal of Virology, 1995-09, Vol.69 (9), p.5791-5797

1995

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Substrate reduction therapy for glycosphingolipid storage disorders

Expert opinion on investigational drugs, 2001-03, Vol.10 (3), p.455-466

2001

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N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120

Journal of Virology, 1996-10, Vol.70 (10), p.7143-7152

1996

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N-butyldeoxynojirimycin reduces growth and ganglioside content of experimental mouse brain tumours

British journal of cancer, 2001-04, Vol.84 (8), p.1107-1114

2001

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Secretion of Human Hepatitis B Virus is Inhibited by the Imino Sugar N- Butyldeoxynojirimycin

Proceedings of the National Academy of Sciences - PNAS, 1994-03, Vol.91 (6), p.2235-2239

1994

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NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress

A master protocol to investigate a novel therapy acetyl-L-leucine for three ultra-rare neurodegenerative diseases: Niemann-Pick type C, the GM2 gangliosidoses, and ataxia telangiectasia

Terror and Greatness: Ivan and Peter as Russian Myths

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

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

Inactivation of the Rb pathway and overexpression of both isoforms of E2F3 are obligate events in bladder tumours with 6p22 amplification

Putative tumour suppressor gene necdin is hypermethylated and mutated in human cancer

Autophagy Induction and Autophagosome Clearance in Neurons: Relationship to Autophagic Pathology in Alzheimer's Disease

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

A Conversation with Maria Stepanova

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis

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

Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy

Prevention of Lysosomal Storage in Tay-Sachs Mice Treated with N-Butyldeoxynojirimycin

In-depth phenotyping for clinical stratification of Gaucher disease

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

N-butyldeoxynojirimycin causes weight loss as a result of appetite suppression in lean and obese mice

New therapeutic prospects for the glycosphingolipid lysosomal storage diseases

The Russophone Literature of Resistance: Introduction

The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding

Substrate reduction therapy for glycosphingolipid storage disorders

N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120

N-butyldeoxynojirimycin reduces growth and ganglioside content of experimental mouse brain tumours

Secretion of Human Hepatitis B Virus is Inhibited by the Imino Sugar N- Butyldeoxynojirimycin

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