Frances Platt

Q: What Schools Are Affiliated With Frances Platt

Frances Platt is affiliated with the following schools: Washington University in St. Louis, University of Bath, Imperial College London, University of Oxford

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Frances Platt

Biology

#9864

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#13119

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Biochemistry

#1750

World Rank

#1885

Historical Rank

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Biology

Frances Platt's Degrees

PhD Biochemistry University of Oxford

Why Is Frances Platt Influential?

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According to Wikipedia, Frances Mary Platt is a British biochemist and pharmacologist who is a professor at the University of Oxford. Her research investigates rare genetic disorders known as lysosomal storage diseases, progressive conditions that lead to neurodegeneration. She was elected Fellow of the Royal Society in 2021.

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Frances Platt's Published Works

Number of citations in a given year to any of this author's works

Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

bcl-2-Immunoglobulin transgenic mice demonstrate extended B cell survival and follicular lymphoproliferation (1989) (1267)
Autophagy Induction and Autophagosome Clearance in Neurons: Relationship to Autophagic Pathology in Alzheimer's Disease (2008) (989)
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium (2008) (755)
Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis (2000) (713)
Lysosomal storage disorders: The cellular impact of lysosomal dysfunction (2012) (517)
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis. (1994) (427)
Targeting glycosylation as a therapeutic approach (2002) (393)
Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide (2007) (377)
Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase (2004) (346)
Lysosomal storage diseases (2018) (335)
Invariant NKT cells reduce the immunosuppressive activity of influenza A virus-induced myeloid-derived suppressor cells in mice and humans. (2008) (334)
Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease. (2011) (326)
Common and Uncommon Pathogenic Cascades in Lysosomal Storage Diseases* (2010) (318)
Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin. (1997) (316)
Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis. (2003) (304)
Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease (2007) (297)
Imino sugars inhibit the formation and secretion of bovine viral diarrhea virus, a pestivirus model of hepatitis C virus: implications for the development of broad spectrum anti-hepatitis virus agents. (1999) (295)
Activation of invariant NKT cells by toll-like receptor 9-stimulated dendritic cells requires type I interferon and charged glycosphingolipids. (2007) (290)
Sphingolipid lysosomal storage disorders (2014) (248)
Inhibition of glycosphingolipid biosynthesis: application to lysosomal storage disorders. (2000) (241)
Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin. (1999) (241)
Deregulated Bcl-2-immunoglobulin transgene expands a resting but responsive immunoglobulin M and D-expressing B-cell population (1990) (239)
Purified TPC Isoforms Form NAADP Receptors with Distinct Roles for Ca2+ Signaling and Endolysosomal Trafficking (2010) (234)
Sustained therapeutic effects of oral miglustat (Zavesca, N-butyldeoxynojirimycin, OGT 918) in type I Gaucher disease (2004) (232)
Normal development and function of invariant natural killer T cells in mice with isoglobotrihexosylceramide (iGb3) deficiency (2007) (227)
Modulation of human natural killer T cell ligands on TLR-mediated antigen-presenting cell activation (2007) (207)
Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses. (2005) (198)
Storage solutions: treating lysosomal disorders of the brain (2005) (197)
Lipids on Trial: The Search for the Offending Metabolite in Niemann‐Pick type C Disease (2010) (190)
Accumulation of Glycosphingolipids in Niemann-Pick C Disease Disrupts Endosomal Transport* (2004) (186)
Secretion of human hepatitis B virus is inhibited by the imino sugar N-butyldeoxynojirimycin. (1994) (179)
Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling. (2004) (175)
N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing. (1994) (173)
High Incidence of Unrecognized Visceral/Neurological Late-onset Niemann-Pick Disease, type C1 Predicted by Analysis of Massively Parallel Sequencing Data Sets (2015) (170)
A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma[S] (2011) (165)
Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals (2007) (160)
Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C (2004) (154)
Emptying the stores: lysosomal diseases and therapeutic strategies (2017) (151)
The alpha-glucosidase inhibitor N-butyldeoxynojirimycin inhibits human immunodeficiency virus entry at the level of post-CD4 binding (1995) (148)
Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases (2006) (145)
RIPK3 as a potential therapeutic target for Gaucher's disease (2014) (144)
Effects of the imino sugar N-butyldeoxynojirimycin on the N-glycosylation of recombinant gp120. (1993) (143)
Inhibition of Calcium Uptake via the Sarco/Endoplasmic Reticulum Ca2+-ATPase in a Mouse Model of Sandhoff Disease and Prevention by Treatment with N-Butyldeoxynojirimycin* (2003) (139)
Therapeutic applications of imino sugars in lysosomal storage disorders. (2003) (139)
N-butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo. (2000) (135)
Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1 (2009) (135)
Activated platelets express IL-1 activity. (1989) (134)
Vesicular and non-vesicular transport feed distinct glycosylation pathways in the Golgi (2013) (133)
Expression of Ca2+-permeable two-pore channels rescues NAADP signalling in TPC-deficient cells (2015) (132)
NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress (2019) (124)
Heat shock protein–based therapy as a potential candidate for treating the sphingolipidoses (2016) (124)
Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases. (2011) (123)
Molecular requirements of imino sugars for the selective control of N-linked glycosylation and glycosphingolipid biosynthesis (2000) (122)
Extensive Glycosphingolipid Depletion in the Liver and Lymphoid Organs of Mice Treated with N-Butyldeoxynojirimycin* (1997) (120)
NSAIDs increase survival in the Sandhoff disease mouse: Synergy with N‐butyldeoxynojirimycin (2004) (118)
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation. (2001) (115)
Impaired antibacterial autophagy links granulomatous intestinal inflammation in Niemann–Pick disease type C1 and XIAP deficiency with NOD2 variants in Crohn's disease (2016) (114)
Mutations in B4GALNT1 (GM2 synthase) underlie a new disorder of ganglioside biosynthesis (2013) (112)
Substrate reduction therapy (2008) (110)
N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with changes in antibody recognition of the V1/V2 region of gp120 (1996) (110)
Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy (2001) (109)
Fetal gene therapy for neurodegenerative disease of infants (2018) (104)
Glucosylceramide modulates membrane traffic along the endocytic pathway Published, JLR Papers in Press, August 16, 2002. DOI 10.1194/jlr.M200232-JLR200 (2002) (101)
Male Germ Cells Require Polyenoic Sphingolipids with Complex Glycosylation for Completion of Meiosis (2008) (99)
The association of Shiga-like toxin with detergent-resistant membranes is modulated by glucosylceramide and is an essential requirement in the endoplasmic reticulum for a cytotoxic effect. (2005) (99)
Intracellular sphingosine releases calcium from lysosomes (2015) (97)
A novel, highly sensitive and specific biomarker for Niemann-Pick type C1 disease (2015) (97)
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis (2002) (96)
Activation of Invariant NKT Cells by the Helminth Parasite Schistosoma mansoni1 (2006) (93)
Macroautophagy Is Not Directly Involved in the Metabolism of Amyloid Precursor Protein* (2010) (92)
Lysosomal Disorders of the Brain (2004) (88)
Treating lysosomal storage disorders: current practice and future prospects. (2009) (82)
N-butyldeoxynojirimycin-mediated inhibition of human immunodeficiency virus entry correlates with impaired gp120 shedding and gp41 exposure (1996) (82)
N‐butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis (2004) (81)
Cyclodextrin alleviates neuronal storage of cholesterol in Niemann-Pick C disease without evidence of detectable blood–brain barrier permeability (2013) (81)
A novel approach to analyze lysosomal dysfunctions through subcellular proteomics and lipidomics: the case of NPC1 deficiency (2017) (80)
Reversible infertility in male mice after oral administration of alkylated imino sugars: A nonhormonal approach to male contraception (2002) (80)
Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease (2019) (79)
Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase (2015) (77)
Identification of novel bile acids as biomarkers for the early diagnosis of Niemann‐Pick C disease (2016) (77)
Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice Published, JLR Papers in Press, February 1, 2005. DOI 10.1194/jlr.M400411-JLR200 (2005) (76)
Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease (2004) (76)
The postacrosomal assembly of sperm head protein, PAWP, is independent of acrosome formation and dependent on microtubular manchette transport. (2007) (76)
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis. (2008) (76)
Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice (2012) (76)
Preparation, biochemical characterization and biological properties of radiolabelled N-alkylated deoxynojirimycins. (2002) (74)
Tyrosinase folding and copper loading in vivo: a crucial role for calnexin and alpha-glucosidase II. (1999) (74)
Inhibition of N-Glycan Processing in B16 Melanoma Cells Results in Inactivation of Tyrosinase but Does Not Prevent Its Transport to the Melanosome* (1997) (73)
New therapeutic prospects for the glycosphingolipid lysosomal storage diseases. (1998) (72)
Therapy of Niemann-Pick disease, type C. (2004) (69)
Improved neuroprotection using miglustat, curcumin and ibuprofen as a triple combination therapy in Niemann–Pick disease type C1 mice (2014) (69)
Disorders of cholesterol metabolism and their unanticipated convergent mechanisms of disease. (2014) (68)
Accumulation of glucosylceramide in murine testis, caused by inhibition of beta-glucosidase 2: implications for spermatogenesis. (2007) (67)
Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker. (2014) (65)
Increased glycosphingolipid levels in serum and aortae of apolipoprotein E gene knockout mice. (2002) (64)
A comparative study on fluorescent cholesterol analogs as versatile cellular reporters[S] (2016) (63)
Substrate reduction therapy in mouse models of the glycosphingolipidoses. (2003) (63)
Diverse Endogenous Antigens for Mouse NKT Cells: Self-Antigens That Are Not Glycosphingolipids (2011) (63)
Endolysosomal calcium regulation and disease. (2010) (62)
Small-molecule therapeutics for the treatment of glycolipid lysosomal storage disorders. (2003) (62)
The sensitivity of murine spermiogenesis to miglustat is a quantitative trait: a pharmacogenetic study (2007) (61)
β-Glucosidase 2 (GBA2) Activity and Imino Sugar Pharmacology* (2013) (61)
Glycosphingolipid levels and glucocerebrosidase activity are altered in normal aging of the mouse brain (2018) (59)
The Yeast P5 Type ATPase, Spf1, Regulates Manganese Transport into the Endoplasmic Reticulum (2013) (58)
High-performance liquid chromatography analysis of ganglioside carbohydrates at the picomole level after ceramide glycanase digestion and fluorescent labeling with 2-aminobenzamide. (2001) (58)
Immune dysfunction in Niemann‐Pick disease type C (2015) (54)
Inhibition of Glucosylceramide Synthase Does Not Reverse Drug Resistance in Cancer Cells* (2004) (52)
Alkylated Imino Sugars, Reversible Male Infertility-Inducing Agents, Do Not Affect the Genetic Integrity of Male Mouse Germ Cells During Short-Term Treatment Despite Induction of Sperm Deformities1 (2005) (52)
N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice (2008) (50)
N -butyldeoxynojirimycin reduces growth and ganglioside content of experimental mouse brain tumours (2001) (47)
Membrane disruption and cytotoxicity of hydrophobic N-alkylated imino sugars is independent of the inhibition of protein and lipid glycosylation. (2003) (47)
Neural Stem Cell Transplantation Benefits a Monogenic Neurometabolic Disorder During the Symptomatic Phase of Disease (2009) (47)
A clinical evaluation of coronary arteriography. (1967) (45)
GM1 ganglioside-independent intoxication by Cholera toxin (2018) (45)
Glycolipid depletion in antimicrobial therapy (2003) (44)
Substrate reduction therapy for glycosphingolipid storage disorders (2001) (43)
Cellular effects of deoxynojirimycin analogues: inhibition of N-linked oligosaccharide processing and generation of free glucosylated oligosaccharides. (2004) (42)
Chemoenzymatic Synthesis of a Phosphorylated Glycoprotein. (2016) (42)
Severe endothelial dysfunction in the aorta of a mouse model of Fabry disease; partial prevention by N-butyldeoxynojirimycin treatment (2007) (40)
Globosides but Not Isoglobosides Can Impact the Development of Invariant NKT Cells and Their Interaction with Dendritic Cells (2012) (39)
AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann–Pick type C1 disease (2018) (39)
Lysosomal defects and storage (2004) (39)
Cellular effects of deoxynojirimycin analogues: uptake, retention and inhibition of glycosphingolipid biosynthesis. (2004) (39)
Neuraminidases 3 and 4 regulate neuronal function by catabolizing brain gangliosides (2017) (39)
FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann‐Pick type C mutant fibroblasts (2017) (39)
Storage diseases: new insights into sphingolipid functions. (2003) (37)
Inhibitors of Glycosphingolipid Biosynthesis (1995) (35)
Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis (2017) (34)
Analysis and isolation of human transferrin receptor using the OKT-9 monoclonal antibody covalently crosslinked to magnetic beads. (1991) (34)
Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway (2016) (34)
Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1. (2014) (32)
Annual severity increment score as a tool for stratifying patients with Niemann-Pick disease type C and for recruitment to clinical trials (2018) (32)
Inhibition of Glycosphingolipid Biosynthesis Does Not Impair Growth or Morphogenesis of the Postimplantation Mouse Embryo (1998) (32)
Critical role of iron in the pathogenesis of the murine gangliosidoses (2009) (31)
Modulation of THP-1 macrophage and cholesterol-loaded foam cell apolipoprotein E levels by glycosphingolipids. (2002) (29)
Lipid-mediated motor-adaptor sequestration impairs axonal lysosome delivery leading to autophagic stress and dystrophy in Niemann-Pick type C. (2021) (29)
Glycosphingolipids in endocytic membrane transport. (2004) (28)
Substrate deprivation: A new therapeutic approach for the glycosphingolipid lysosomal storage diseases (2000) (28)
N-Butyl-l-deoxynojirimycin (l-NBDNJ): Synthesis of an Allosteric Enhancer of α-Glucosidase Activity for the Treatment of Pompe Disease. (2017) (27)
N‐butyldeoxynojirimycin causes weight loss as a result of appetite suppression in lean and obese mice (2007) (27)
New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases. (2003) (27)
An Inducible Mouse Model of Late Onset Tay–Sachs Disease (2002) (27)
Long-term non-hormonal male contraception in mice using N-butyldeoxynojirimycin. (2006) (27)
Biomarkers for disease progression and AAV therapeutic efficacy in feline Sandhoff disease (2015) (26)
CD1d presentation of glycolipids (2008) (25)
Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms (2020) (25)
Inhibition of glycogen breakdown by imino sugars in vitro and in vivo. (2004) (24)
A galactopyranose analogue of hydantocidin (1996) (23)
Silencing the porcine iGb3s gene does not affect Galα3Gal levels or measures of anticipated pig‐to‐human and pig‐to‐primate acute rejection (2016) (22)
c-Abl Inhibition Activates TFEB and Promotes Cellular Clearance in a Lysosomal Disorder (2020) (21)
GM1 Gangliosidosis—A Mini-Review (2021) (21)
Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease (2016) (21)
Acetyl-leucine slows disease progression in lysosomal storage disorders (2020) (21)
Bridging the age spectrum of neurodegenerative storage diseases. (2015) (20)
A Novel Mouse Model of a Patient Mucolipidosis II Mutation Recapitulates Disease Pathology* (2014) (20)
Glycolipid receptor depletion as an approach to specific antimicrobial therapy. (2006) (20)
Synthesis of 1,5-dideoxy-3-O-(α-D-mannopyranosyl)-1,5-imino-D-mannitol and 1,5-dideoxy-3-O-(α-D-glucopyranosyl)-1,5-imino-D-mannitol: Powerful inhibitors of endomannosidase (1993) (20)
Modulation of cell-surface transferrin receptor by the imino sugar N-butyldeoxynojirimycin. (1992) (19)
The metabolism of glucocerebrosides - From 1965 to the present. (2017) (19)
Systemic AAV9 gene therapy using the synapsin I promoter rescues a mouse model of neuronopathic Gaucher disease but with limited cross-correction potential to astrocytes (2020) (18)
Invariant natural killer T cells are not affected by lysosomal storage in patients with Niemann‐Pick disease type C (2012) (17)
Unexpected differences in the pharmacokinetics of N-acetyl-DL-leucine enantiomers after oral dosing and their clinical relevance (2019) (17)
Inhibition of α-Glucosidases I and II Increases the Cell Surface Expression of Functional Class A Macrophage Scavenger Receptor (SR-A) by Extending Its Half-life* (2004) (17)
MRS reveals additional hexose N‐acetyl resonances in the brain of a mouse model for Sandhoff disease (2005) (17)
Drug‐induced increase in lysobisphosphatidic acid reduces the cholesterol overload in Niemann–Pick type C cells and mice (2019) (17)
BETA-GLUCOSIDASE 2 ( GBA 2 ) ACTIVITY AND IMINO SUGAR PHARMACOLOGY (2013) (16)
High-performance cation-exchange chromatography and pulsed amperometric detection for the separation, detection, and quantitation of N-alkylated imino sugars in biological samples. (2000) (16)
Beneficial Effects of Acetyl-DL-Leucine (ADLL) in a Mouse Model of Sandhoff Disease (2020) (16)
NMR analysis reveals significant differences in the plasma metabolic profiles of Niemann Pick C1 patients, heterozygous carriers, and healthy controls (2017) (15)
Guidelines for incorporating scientific knowledge and practice on rare diseases into higher education: neuronal ceroid lipofuscinoses as a model disorder. (2015) (15)
Staying in touch with the endocytic network: The importance of contacts for cholesterol transport (2020) (15)
Carbohydrate receptor depletion as an antimicrobial strategy for prevention of urinary tract infection. (2001) (15)
Aberrant control of galactosyltransferase in peripheral B lymphocytes and Epstein-Barr virus transformed B lymphoblasts from patients with rheumatoid arthritis. (1993) (15)
NAADP Receptors with Distinct Roles for Ca 2 + Signaling and Endolysosomal Trafficking (2010) (14)
1H NMR-Linked Urinary Metabolic Profiling of Niemann-Pick Class C1 (NPC1) Disease: Identification of Potential New Biomarkers using Correlated Component Regression (CCR) and Genetic Algorithm (GA) Analysis Strategies (2014) (14)
Effects of miglustat treatment in a patient affected by an atypical form of Tangier disease (2014) (14)
Sterile activation of invariant natural killer T cells by ER-stressed antigen-presenting cells (2019) (14)
Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity (2020) (14)
Medicinal use of iminosugars (2008) (14)
TLR9-mediated dendritic cell activation uncovers mammalian ganglioside species with specific ceramide backbones that activate invariant natural killer T cells (2019) (13)
Glycosylation: disease targets and therapy. (2005) (12)
New developments in treating glycosphingolipid storage diseases. (2005) (12)
Lysosomal Disorders of the Brain: Recent Advances in Molecular and Cellular Pathogenesis and Treatment (2004) (12)
Drug-induced increase in lysobisphosphatidic acid reduces the cholesterol overload in Niemann-Pick type C cells and mice (2018) (12)
Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice (2010) (12)
Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3−/− Mice (2019) (11)
Hepatic metabolic response to restricted copper intake in a Niemann-Pick C murine model. (2014) (11)
Haematopoietic Stem Cell Transplantation Arrests the Progression of Neurodegenerative Disease in Late-Onset Tay-Sachs Disease. (2017) (11)
Metabolomic Studies of Lipid Storage Disorders, with Special Reference to Niemann-Pick Type C Disease: A Critical Review with Future Perspectives (2020) (10)
Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis (2018) (10)
Investigating the Mechanism of Cyclodextrins in the Treatment of Niemann-Pick Disease Type C Using Crosslinked 2-Hydroxypropyl-β-cyclodextrin. (2020) (10)
1H NMR-Linked Metabolomics Analysis of Liver from a Mouse Model of NP-C1 Disease. (2016) (10)
Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases (2017) (10)
Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1 (2020) (10)
Imino sugar therapy for type 1 Gaucher disease. (2000) (10)
NPC1 Deficiency in Mice is Associated with Fetal Growth Restriction, Neonatal Lethality and Abnormal Lung Pathology (2019) (9)
Age-related gait standards for healthy children and young people: the GOS-ICH paediatric gait centiles (2019) (9)
Preferential Coupling of the NAADP Pathway to Exocytosis in T-Cells. (2015) (9)
Current methods to analyze lysosome morphology, positioning, motility and function (2022) (9)
Mechanistic convergence and shared therapeutic targets in Niemann‐Pick disease (2019) (9)
Differential sensitivity of mouse strains to an N-alkylated imino sugar: glycosphingolipid metabolism and acrosome formation. (2008) (9)
An iPSC model of hereditary sensory neuropathy-1 reveals L-serine-responsive deficits in neuronal ganglioside composition and axoglial interactions (2021) (8)
Monoclonal antibodies specific for novel murine cell surface markers define subpopulations of germinal center cells. (1992) (8)
Genetic background modifies phenotypic severity and longevity in a mouse model of Niemann-Pick disease type C1. (2020) (8)
Sandhoff Disease: Improvement of Gait by Acetyl-DL-Leucine: A Case Report (2020) (8)
Selective estrogen receptor modulators (SERMs) affect cholesterol homeostasis through the master regulators SREBP and LXR. (2021) (8)
Acetylation turns leucine into a drug by membrane transporter switching (2021) (7)
62. Abnormal lysosomal calcium homeostasis in mucolipidosis type IV (2008) (7)
An anecdotal report by an Oxford basic neuroscientist: effects of acetyl-dl-leucine on cognitive function and mobility in the elderly (2016) (7)
Author Correction: Lysosomal storage diseases (2018) (7)
Ultrastructural changes in the Golgi apparatus and secretory granules of HL-60 cells treated with the imino sugar N-butyldeoxynojirimycin. (1997) (7)
Lysosomes and lysosomal diseases (2015) (7)
Vesicle cholesterol controls exocytotic fusion pore. (2021) (7)
Lucerastat, an iminosugar for substrate reduction therapy in Fabry disease: preclinical evidence (2017) (7)
Glycomimetic affinity-enrichment proteomics identifies partners for a clinically-utilized iminosugar. (2013) (7)
Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease (2016) (6)
Measuring relative lysosomal volume for monitoring lysosomal storage diseases. (2015) (6)
Glycosphingolipid metabolism and its role in ageing and Parkinson’s disease (2021) (6)
Accumulation of Glucosylceramide in Murine Testis, Caused by Inhibition of β-Glucosidase 2 (2007) (6)
Transcriptome of HPβCD-treated Niemann-pick disease type C1 cells highlights GPNMB as a biomarker for therapeutics. (2021) (6)
Publisher Correction: Lysosomal storage diseases (2019) (5)
Glycosphingolipid Changes in Plasma in Parkinson's Disease Independent of Glucosylceramide Levels (2022) (5)
Synthesis and Study of Multifunctional Cyclodextrin–Deferasirox Hybrids (2019) (5)
Unbiased yeast screens identify cellular pathways affected in Niemann–Pick disease type C (2020) (5)
Imaging of changes in copper trafficking and redistribution in a mouse model of Niemann-Pick C disease using positron emission tomography (2019) (5)
Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity (2021) (5)
Lipid-mediated impairment of axonal lysosome transport contributing to autophagic stress (2021) (4)
Urinary excretion and metabolism of miglustat and valproate in patients with Niemann-Pick type C1 disease: One- and two-dimensional solution-state (1)H NMR studies. (2016) (4)
Substrate reduction therapy in Sandhoff disease: Evidence for improvement in nervous function in patients treated with miglustat (2006) (4)
Molecular basis for a new bovine model of Niemann-Pick type C disease (2020) (4)
Glycosphingolipid depletion in PC12 cells using iminosugars protects neuronal membranes from anti-ganglioside antibody mediated injury (2008) (4)
Introduction: Glycosphingolipids in cell biology and disease. (2004) (3)
Pseudogenes limit the identification of novel common transcripts generated by their parent genes (2022) (3)
A new surrogate marker for CNS pathology in Niemann-Pick disease type C? (2009) (3)
Glucosamine amends CNS pathology in mucopolysaccharidosis IIIC mouse expressing misfolded HGSNAT (2022) (3)
International consensus on clinical severity scale use in evaluating Niemann–Pick disease Type C in paediatric and adult patients: results from a Delphi Study (2021) (2)
Vesicle Cholesterol Controls Exocytotic Fusion Pore (2021) (2)
Redistribution of cholesterol from vesicle to plasmalemma controls fusion pore geometry (2020) (2)
61. Smith-Lemli-Opitz syndrome: A closet lysosomal storage disease hiding within an inheritable metabolic disorder of cholesterol biosynthesis (2008) (2)
Diaphragm — Deltoid synkinesis with myelographic demonstration of the cause (1971) (2)
Testing combinatorial therapies for juvenile Batten disease: Lysosome (2018) (2018) (2)
Identification of new biomarkers suitable for an early diagnosis of Niemann-Pick C1 (2016) (2)
Upregulating beta-hexosaminidase activity in rodents prevents alpha-synuclein lipid associations and protects dopaminergic neurons from alpha-synuclein-mediated neurotoxicity (2020) (1)
Miglustat: A Viewpoint by Frances M. Platt and Terry D. Butters (2003) (1)
Correlation of age of onset and clinical severity in Niemann–Pick disease type C1 with lysosomal abnormalities and gene expression (2022) (1)
"Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice" (vol 46, pg 744, 2004) (2005) (1)
Neural stem cell therapy in lysosomal storage disorders (2007) (1)
Heat shock protein amplification improves cerebellar myelination in the Npc1nih mouse model (2022) (1)
Author response: Intracellular sphingosine releases calcium from lysosomes (2015) (1)
Ultrastructural changes in the Golgi apparatus and secretory granules of HL‐60 cells treated with the imino sugar N‐butyldeoxynojirimycin (1997) (1)
Miglustat: A Viewpoint (2003) (1)
Relative Acidic Compartment Volume as a Biomarker (2014) (1)
A modified density gradient proteomic-based method to analyze endolysosomal proteins in cardiac tissue (2021) (1)
Development of gene therapy for Niemann-Pick Type C disease (2015) (1)
Defective iron homeostasis and hematological abnormalities in Niemann-Pick disease type C1 (2022) (1)
Defective platelet function in Niemann‐Pick disease type C1 (2020) (1)
Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease (2020) (1)
Acetylation of L-leucine switches its carrier from the L-amino acid transporter (LAT) to organic anion transporters (OAT) (2020) (1)
Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C [version 1; referees: 2 approved, 1 not approved] (2017) (1)
substrate deprivation therapy and bone marrow transplantation Enhanced survival in Sandhoff disease mice receiving a combination of (2013) (1)
Inhibition of phago-lysosome fusion and foam cell formation by Mycobacterium bovis BCG induces a Niemann-Pick type C1 like phenotype (2009) (1)
Heat shock protein-based therapy for sphingolipidoses (2017) (1)
Defective iron homeostasis and hematological abnormalities in Niemann-Pick disease type C1. (2022) (1)
Anti-TNF therapy for inflammatory bowel disease in patients with neurodegenerative Niemann-Pick disease Type C [version 1; peer review: awaiting peer review] (2022) (0)
Glycobiology – discovery, diagnosticsand drugs (2007) (0)
AAV9 Intracerebroventricular Gene Therapy Improves Lifespan and Normalises Long-Term Locomotor Behaviour in a Mouse Model of Niemann-Pick Type C1 Disease (2018) (0)
The use of long chain N-alkyl derivatives of deoxynojirimycin for the manufacture of a medicament for the treatment of diseases glycolipidophobnings (1999) (0)
Impaired Fc-gamma and complement receptor mediated phagocytosis in Niemann-Pick disease type C (NPC) macrophages (2016) (0)
Pathogenic mycobacteria achieve cellular persistence via lipid-mediated inhibition of the Niemann-Pick disease type C pathway (2014) (0)
Invariant NKT cells metabolically adapt to the acute myeloid leukaemia environment (2022) (0)
Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease (2019) (0)
c-Abl Inhibition Activates TFEB: A Novel Way To Promote Cellular Clearance in Lysosomal Disorders (2020) (0)
Lipid mediated inhibition of Niemann-Pick C1 protein is an evolutionary conserved feature of multiple Mycobacterium lineages and non-tubercular mycobacteria (2021) (0)
Defective iron homeostasis in lysosomal storage diseases (2013) (0)
Synthesis of 1,5-Dideoxy-3-O-(α-D-mannopyranosyl)-1,5-imino-D- mannitol (XI) and 1,5-Dideoxy-3-O-(α-D-glucopyranosyl)-1,5-imino- D-mannitol (IX): Powerful Inhibitors of Endomannosidase. (1994) (0)
Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences: 358 (1433) (2003) (0)
The Molecular Basis of the Regulation of the TMEM16A Ca2+-Activated Cl- Channel by the Lysosomal NPC1 Protein (2021) (0)
Use of long chain N-alkyl derivatives of deoxynojirimycin for the manufacture of a medicament for the treatment of accumulation with glycolipids related diseases (1999) (0)
Emptying the stores: lysosomaldiseases and therapeutic strategies (2017) (0)
Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity (2020) (0)
Effects of substrate deprivation therapy on brain lipids in neonatal GML storage disease mice (2003) (0)
The Role of the GlcCer Transfer Protein FAPP2 at the Golgi Complex (2008) (0)
Combined deficiencies of endosomal ganglioside neuraminidases 3 and 4 in mice cause lysosomal GM3 gangliosidosis (2017) (0)
Glycolipid receptordepletionas anapproach to speci ¢ c antimicrobial therapy (2006) (0)
Inhibition of NPC1 function by cholesterol precursors in Smith–Lemli–Opitz syndrome induces an intracellular phenotype identical to Niemann–Pick C disease (2009) (0)
Helminth Parasite Schistosoma mansoni Activation of Invariant NKT Cells by the (2013) (0)
Dysregulation of cholesterol homeostasis (2015) (0)
Release of acidic store calcium is required for effective priming of the NLRP3 inflammasome (2022) (0)
Substrate deprivation therapy reduces brain ganglioside content and GM1 in neonatal storage disease mice (2002) (0)
Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis (2017) (0)
Targeting GLB1 in mice by CRISPR/Cas9 genome editing: Establishing a novel model for type II GM1 gangliosidosis (2020) (0)
SU61 ASSESSING BEHAVIOR AND ANXIETY IN THE DHCR7Δ3-5/T93M MOUSE MODEL OF SMITH-LEMLI-OPITZ SYNDROME (2019) (0)
Changes in glial cell function in Niemann-Pick type C disease as therapeutic targets (2017) (0)
derivatives using deoxygalactonojirimycin to inhibition of glycolipid synthesis (1994) (0)
Oral Glucosamine Ameliorates Aggravated Neurological Phenotype in Mucopolysaccharidosis III Type C Mouse Model Expressing Misfolded HGSNAT Variant (2021) (0)
142. SOAR collaborative: Progress toward therapy for a lysosomal disease through an academic research–family partnership (2010) (0)
One- and two-dimensional NMR investigations of the urinary excretion and metabolism of miglustat and valproate in patients with Niemann-Pick disease type C1 (2016) (0)
The apo E gene knockout mouse as a model to study the potential atherogenic properties of glycosphingolipids (2003) (0)
Selective Inhibition of the Non-lysosomal Glucosylceramidase Gba2 and Elevation of Glucosylceramide in vivo: Consequences for Male Germ Cell Development (2008) (0)
This includes enzyme replacement therapy , bone marrow transplantation , gene therapy and stem cell therapy (2008) (0)
Compartmentalisation proteomics revealed endolysosomal protein network changes in a goat model of atrial fibrillation (2023) (0)
Glb1 knockout mouse model shares natural history with type II GM1 gangliosidosis patients. (2023) (0)
Inhibition of the Niemann-Pick C1 protein is a conserved feature of multiple strains of pathogenic mycobacteria (2022) (0)
59. Inhibition of the nicotinic acid adenine dinucleotide phosphate receptor induces a novel lysosomal storage disease (2008) (0)
STUDIES ON THE MECHANISM OF ACTION OF N-BUTYL-DEOXYNOJIRIMYCIN AS AN INHIBITOR OF HIV REPLICATION (1995) (0)
N-butyldeoxygalactonojirimycin reduces postnatal cerebellar ganglioside content in a mouse model of GM1 gangliosidosis (2004) (0)
Use of n-alkyl derivatives of deoxynojirimycin long chain for the manufacture of a medicament for the treatment of diseases glycolipid storage. (1999) (0)
Pharmacological impairment of acrosome formation and sperm head morphogenesis reveals essential role of glycosphingolipids in spermiogenesis (2002) (0)
Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease (2020) (0)
Neuraminidases 3 and 4 direct neuronal development and function by reshaping the composition of brain gangliosides (2017) (0)
LYSOSOMAL SPHINGOSINE STORAGE INDUCED BY MYCOLIC ACID MEDIATED INHIBITION OF THE NPC1 PROTEIN IS CENTRAL TO Mycobacterium tuberculosis INDUCED PATHOGENESIS (2010) (0)
FULL PAPER Synthesis and Study of Novel Multifunctional Cyclodextrin- Deferasirox Hybrids (2019) (0)
Defective macrophage phagocytosis and particle clearance in Niemann-Pick disease type C1 mice (2017) (0)
An MRI technique for the quantification of atherosclerotic lesions in the ApoE knockout mouse (2002) (0)
Front Cover: Synthesis and Study of Multifunctional Cyclodextrin–Deferasirox Hybrids (ChemMedChem 16/2019) (2019) (0)
Glycolipids in cell biology and medicine - Introduction (2003) (0)
A method for the treatment of CNS-diseases involving lysosomal storage (1998) (0)
Analysis of stored oligosaccharides in a mouse model of a glycolipid storage (Sandhoff) disease (2003) (0)
Anti-TNF therapy for inflammatory bowel disease in patients with neurodegenerative Niemann-Pick disease Type C (2022) (0)
Schistosoma mansoni Helminth Parasite Activation of Invariant NKT Cells by the (2006) (0)
Correction: Molecular basis for a new bovine model of Niemann-Pick type C disease (2021) (0)
Deficiency in Sphingolipids Has No Effect on Self-antigen Presentation to Invariant NKT Cells (134.17) (2009) (0)
Anti-TNF therapy for inflammatory bowel disease in patients with neurodegenerative Niemann-Pick disease Type C [version 1; peer review: 1 approved] (2022) (0)
Cardiac Atrial Compartmentalisation Proteomics: A Modified Density Gradient Method to Analyse Endo-lysosomal Proteins (2021) (0)
ALTERED AUTOPHAGY AND APP METABOLISM IN GLYCOSPHINGOLIPID STORAGE DISEASES (2011) (0)
Niemann–Pick type C1—A primary sphingosine storage disease? (2007) (0)
77. Glycosphingolipid profiling of tissues from mouse models of human lysosomal storage disorders (2008) (0)
Miglustat: profile report (2012) (0)
GlycosphingolipidsNKT Cells: Self-Antigens That Are Not Diverse Endogenous Antigens for Mouse (2010) (0)
Cholera toxin binds to LewisX and fucosylated glycoproteins play a functional role in human intestinal cell intoxication (2017) (0)
Inhibition of Glycosphingolipid Biosynthesis: Application to Lysosomal Storage Disorders (2001) (0)
Porcine iGb3s gene silencing provides minimal benefit for clinical xenotransplantation (2016) (0)
Evaluation of new therapies in Niemann-Pick type C disease (2014) (0)
NMR analysis reveals significant differences in the plasma metabolic profiles of Niemann Pick C1 patients, heterozygous carriers, and healthy controls (2017) (0)
Human GM3 synthase deficiency: A novel form of hereditary childhood epilepsy (2005) (0)
Glycosphingolipid metabolism and its role in ageing and Parkinson’s disease (2021) (0)
Search for the Offending Metabolite in NPC Disease Cholesterol Sphingomyelin Glucosylceramide Sphingosine ( GSLs ) Brain 0 X 0 X 3 X Liver 4 X 4 X 2 X 12 X 4 X (2010) (0)
Towards Clinical Progress in the Mucopolysaccharidoses (2007) (0)
The regulation of postsynaptic GABAA receptor signalling in epilepsy (2013) (0)
Substrate reduction therapy in lysosomal storage diseases: a clarification (2004) (0)
Imaging of the Pla- centa: A Multimodal- (2009) (0)
Imaging of changes in copper trafficking and redistribution in a mouse model of Niemann-Pick C disease using positron emission tomography (2019) (0)
Fetal gene therapy for neurodegenerative disease of infants (2018) (0)
FULL Synthesis and Study of Novel Multifunctional Cyclodextrin-Deferasirox Hybrids (2020) (0)
Anthony Cascades in Lysosomal Storage Diseases Common and Uncommon Pathogenic Minireviews : (2010) (0)
A Galactopyranose Analogue of Hydantocidin. (2010) (0)
Therapeutic assessment of substrate deprivation using NB-DGJ in GM1 storage disease mice (2003) (0)
Human GM3 synthase deficiency: Autosomal recessive infantile onset symptomatic epilepsy syndrome. (2004) (0)
A Mouse Systems Genetics Approach Reveals Common and Uncommon Genetic Modifiers of Hepatic Lysosomal Enzyme Activities and Glycosphingolipids (2023) (0)
Elucidating the mechanism of cyclodextrins in the treatment of Niemann-Pick Disease Type C using crosslinked 2-hydroxypropyl-β-cyclodextrin (2020) (0)
Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C (2017) (0)

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