Published in J Biol Chem on April 12, 2004
Identification of an intracellular site of prion conversion. PLoS Pathog (2009) 1.61
Niemann-Pick C2 (NPC2) and intracellular cholesterol trafficking. Biochim Biophys Acta (2009) 1.54
Monitoring autophagy in lysosomal storage disorders. Methods Enzymol (2009) 1.40
Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models. Mol Genet Metab (2010) 1.35
Secondary lipid accumulation in lysosomal disease. Biochim Biophys Acta (2008) 1.30
Lysosomal lipid storage diseases. Cold Spring Harb Perspect Biol (2011) 1.26
Regulation of sterol transport between membranes and NPC2. Biochemistry (2008) 1.23
New therapies in the management of Niemann-Pick type C disease: clinical utility of miglustat. Ther Clin Risk Manag (2009) 1.23
Alkaline methanolysis of lipid extracts extends shotgun lipidomics analyses to the low-abundance regime of cellular sphingolipids. Anal Biochem (2007) 1.22
Resistance to alkyl-lysophospholipid-induced apoptosis due to downregulated sphingomyelin synthase 1 expression with consequent sphingomyelin- and cholesterol-deficiency in lipid rafts. Biochem J (2007) 1.18
Mucolipin 1: endocytosis and cation channel--a review. Pflugers Arch (2004) 1.16
Lysosomal storage diseases--the horizon expands. Nat Rev Neurol (2013) 1.15
The National Niemann-Pick Type C1 Disease Database: correlation of lipid profiles, mutations, and biochemical phenotypes. J Lipid Res (2009) 1.12
Phosphatidylserine dynamics in cellular membranes. Mol Biol Cell (2012) 1.10
Annexin A6-induced alterations in cholesterol transport and caveolin export from the Golgi complex. Traffic (2007) 1.05
NPC1 late endosomes contain elevated levels of non-esterified ('free') fatty acids and an abnormally glycosylated form of the NPC2 protein. Biochem J (2005) 1.03
Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion. J Lipid Res (2010) 1.01
Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice. Neurobiol Dis (2011) 1.00
Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin. J Lipid Res (2014) 0.96
GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice. J Lipid Res (2008) 0.91
Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain. J Lipid Res (2011) 0.90
Glycolipid trafficking in Drosophila undergoes pathway switching in response to aberrant cholesterol levels. Mol Biol Cell (2010) 0.89
Intracellular sphingosine releases calcium from lysosomes. Elife (2015) 0.88
Bone-marrow-derived mesenchymal stem cells promote proliferation and neuronal differentiation of Niemann-Pick type C mouse neural stem cells by upregulation and secretion of CCL2. Hum Gene Ther (2013) 0.88
A review of lipidomic technologies applicable to sphingolipidomics and their relevant applications. Eur J Lipid Sci Technol (2009) 0.88
Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2. J Lipid Res (2014) 0.88
A fluorescent glycolipid-binding peptide probe traces cholesterol dependent microdomain-derived trafficking pathways. PLoS One (2008) 0.86
Cholesterol in Niemann-Pick Type C disease. Subcell Biochem (2010) 0.85
Abnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategy. Proc Natl Acad Sci U S A (2013) 0.82
Niemann-Pick C1 affects the gene delivery efficacy of degradable polymeric nanoparticles. ACS Nano (2014) 0.81
The potential of histone deacetylase inhibitors in Niemann - Pick type C disease. FEBS J (2013) 0.80
Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice. J Inherit Metab Dis (2010) 0.80
Lipid rafts: dream or reality for cholesterol transporters? Eur Biophys J (2007) 0.79
Treatment of Human Fibroblasts Carrying NPC1 Missense Mutations with MG132 Leads to an Improvement of Intracellular Cholesterol Trafficking. JIMD Rep (2011) 0.79
My journey into the world of sphingolipids and sphingolipidoses. Proc Jpn Acad Ser B Phys Biol Sci (2012) 0.79
Necroptosis in Niemann-Pick disease, type C1: a potential therapeutic target. Cell Death Dis (2016) 0.77
Increased Regenerative Capacity of the Olfactory Epithelium in Niemann-Pick Disease Type C1. Int J Mol Sci (2017) 0.75
Trifunctional lipid probes for comprehensive studies of single lipid species in living cells. Proc Natl Acad Sci U S A (2017) 0.75
Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase. J Lipid Res (2017) 0.75
A novel approach to analyze lysosomal dysfunctions through subcellular proteomics and lipidomics: the case of NPC1 deficiency. Sci Rep (2017) 0.75
Prospective Turkish Cohort Study to Investigate the Frequency of Niemann-Pick Disease Type C Mutations in Consanguineous Families with at Least One Homozygous Family Member. Mol Diagn Ther (2017) 0.75
The broadly neutralizing anti-human immunodeficiency virus type 1 antibody 2G12 recognizes a cluster of alpha1-->2 mannose residues on the outer face of gp120. J Virol (2002) 7.82
Antibody domain exchange is an immunological solution to carbohydrate cluster recognition. Science (2003) 6.68
The impact of glycosylation on the biological function and structure of human immunoglobulins. Annu Rev Immunol (2007) 4.96
Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease. J Neurosci (2008) 4.63
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nat Med (2008) 3.86
Biochemistry and molecular biology of gelatinase B or matrix metalloproteinase-9 (MMP-9). Crit Rev Biochem Mol Biol (2002) 3.53
Statistical analysis of the protein environment of N-glycosylation sites: implications for occupancy, structure, and folding. Glycobiology (2003) 3.19
Glycosphingolipid synthesis requires FAPP2 transfer of glucosylceramide. Nature (2007) 3.13
Exploiting the defensive sugars of HIV-1 for drug and vaccine design. Nature (2007) 3.01
Proposal for a standard system for drawing structural diagrams of N- and O-linked carbohydrates and related compounds. Proteomics (2009) 2.93
Comparison of the methods for profiling glycoprotein glycans--HUPO Human Disease Glycomics/Proteome Initiative multi-institutional study. Glycobiology (2007) 2.71
Use of targeted glycoproteomics to identify serum glycoproteins that correlate with liver cancer in woodchucks and humans. Proc Natl Acad Sci U S A (2005) 2.70
The hepatitis C virus p7 protein forms an ion channel that is inhibited by long-alkyl-chain iminosugar derivatives. Proc Natl Acad Sci U S A (2003) 2.64
Structural characterization of the 1918 influenza virus H1N1 neuraminidase. J Virol (2008) 2.63
Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. Nat Med (2007) 2.57
Envelope glycans of immunodeficiency virions are almost entirely oligomannose antigens. Proc Natl Acad Sci U S A (2010) 2.51
Invariant NKT cells reduce the immunosuppressive activity of influenza A virus-induced myeloid-derived suppressor cells in mice and humans. J Clin Invest (2008) 2.46
Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase. Nat Genet (2004) 2.45
Ovarian cancer is associated with changes in glycosylation in both acute-phase proteins and IgG. Glycobiology (2007) 2.45
Glycoprotein structural genomics: solving the glycosylation problem. Structure (2007) 2.40
Normal development and function of invariant natural killer T cells in mice with isoglobotrihexosylceramide (iGb3) deficiency. Proc Natl Acad Sci U S A (2007) 2.24
Contrasting IgG structures reveal extreme asymmetry and flexibility. J Mol Biol (2002) 2.10
The mannose receptor mediates dengue virus infection of macrophages. PLoS Pathog (2008) 2.09
Ceramide: second messenger or modulator of membrane structure and dynamics? Biochem J (2003) 2.06
Conformational studies of oligosaccharides and glycopeptides: complementarity of NMR, X-ray crystallography, and molecular modelling. Chem Rev (2002) 2.02
Targeting glycosylation as a therapeutic approach. Nat Rev Drug Discov (2002) 1.95
The glycan shield of HIV is predominantly oligomannose independently of production system or viral clade. PLoS One (2011) 1.95
Modulation of human natural killer T cell ligands on TLR-mediated antigen-presenting cell activation. Proc Natl Acad Sci U S A (2007) 1.92
HPLC-based analysis of serum N-glycans on a 96-well plate platform with dedicated database software. Anal Biochem (2007) 1.92
Altered glycosylation pattern allows the distinction between prostate-specific antigen (PSA) from normal and tumor origins. Glycobiology (2003) 1.91
Reversible infertility in male mice after oral administration of alkylated imino sugars: a nonhormonal approach to male contraception. Proc Natl Acad Sci U S A (2002) 1.88
Common and uncommon pathogenic cascades in lysosomal storage diseases. J Biol Chem (2010) 1.85
Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals. Proc Natl Acad Sci U S A (2007) 1.84
Purified TPC isoforms form NAADP receptors with distinct roles for Ca(2+) signaling and endolysosomal trafficking. Curr Biol (2010) 1.84
Emerging principles for the therapeutic exploitation of glycosylation. Science (2014) 1.83
Antiviral effects of an iminosugar derivative on flavivirus infections. J Virol (2002) 1.80
A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma. J Lipid Res (2011) 1.76
Secretory IgA N- and O-glycans provide a link between the innate and adaptive immune systems. J Biol Chem (2003) 1.68
Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease. Biochem J (2011) 1.68
Storage solutions: treating lysosomal disorders of the brain. Nat Rev Neurosci (2005) 1.65
GlycoBase and autoGU: tools for HPLC-based glycan analysis. Bioinformatics (2008) 1.64
Different glycan structures in prostate-specific antigen from prostate cancer sera in relation to seminal plasma PSA. Glycobiology (2005) 1.60
Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases. J Exp Med (2006) 1.57
A strategy to reveal potential glycan markers from serum glycoproteins associated with breast cancer progression. Glycobiology (2008) 1.55
Novel serum biomarker candidates for liver fibrosis in hepatitis C patients. Clin Chem (2007) 1.52
Genes contributing to prion pathogenesis. J Gen Virol (2008) 1.51
Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C. Neurobiol Dis (2004) 1.50
Lipids on trial: the search for the offending metabolite in Niemann-Pick type C disease. Traffic (2010) 1.49
Antiviral effects of amantadine and iminosugar derivatives against hepatitis C virus. Hepatology (2007) 1.49
Structural and quantitative analysis of N-linked glycans by matrix-assisted laser desorption ionization and negative ion nanospray mass spectrometry. Anal Biochem (2008) 1.49
Human serum IgM glycosylation: identification of glycoforms that can bind to mannan-binding lectin. J Biol Chem (2005) 1.47
The 3-dimensional structure of a hepatitis C virus p7 ion channel by electron microscopy. Proc Natl Acad Sci U S A (2009) 1.44
Detailed structural analysis of N-glycans released from glycoproteins in SDS-PAGE gel bands using HPLC combined with exoglycosidase array digestions. Methods Mol Biol (2006) 1.43
Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling. Anal Biochem (2004) 1.42
Inhibition of mammalian glycan biosynthesis produces non-self antigens for a broadly neutralising, HIV-1 specific antibody. J Mol Biol (2007) 1.41
Reactivity-based one-pot synthesis of oligomannoses: defining antigens recognized by 2G12, a broadly neutralizing anti-HIV-1 antibody. Angew Chem Int Ed Engl (2004) 1.41
Molecular mechanism of lipopeptide presentation by CD1a. Immunity (2005) 1.41
Polysaccharide mimicry of the epitope of the broadly neutralizing anti-HIV antibody, 2G12, induces enhanced antibody responses to self oligomannose glycans. Glycobiology (2010) 1.39
Differential proteome analysis of TRAP-activated platelets: involvement of DOK-2 and phosphorylation of RGS proteins. Blood (2003) 1.39
The hemopexin and O-glycosylated domains tune gelatinase B/MMP-9 bioavailability via inhibition and binding to cargo receptors. J Biol Chem (2006) 1.36
Anti-cancer alkyl-lysophospholipids inhibit the phosphatidylinositol 3-kinase-Akt/PKB survival pathway. Anticancer Drugs (2003) 1.35
Increased levels of galactose-deficient anti-Gal immunoglobulin G in the sera of hepatitis C virus-infected individuals with fibrosis and cirrhosis. J Virol (2007) 1.33
Detailed glycan analysis of serum glycoproteins of patients with congenital disorders of glycosylation indicates the specific defective glycan processing step and provides an insight into pathogenesis. Glycobiology (2003) 1.32
Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses. Glycobiology (2005) 1.31
Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases. Cell Calcium (2011) 1.30
Glycosylation changes on serum glycoproteins in ovarian cancer may contribute to disease pathogenesis. Dis Markers (2008) 1.27
An analytical and structural database provides a strategy for sequencing O-glycans from microgram quantities of glycoproteins. Anal Biochem (2002) 1.25
Mannan binding lectin and its interaction with immunoglobulins in health and in disease. Immunol Lett (2006) 1.21
Towards complete analysis of the platelet proteome. Proteomics (2002) 1.21
Crystallographic, thermodynamic, and molecular modeling studies of the mode of binding of oligosaccharides to the potent antiviral protein griffithsin. Proteins (2007) 1.20
Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiol Dis (2005) 1.20
Diverse endogenous antigens for mouse NKT cells: self-antigens that are not glycosphingolipids. J Immunol (2010) 1.20
Human follicular lymphoma cells contain oligomannose glycans in the antigen-binding site of the B-cell receptor. J Biol Chem (2006) 1.19
Male germ cells require polyenoic sphingolipids with complex glycosylation for completion of meiosis: a link to ceramide synthase-3. J Biol Chem (2008) 1.18
Inhibition of hybrid- and complex-type glycosylation reveals the presence of the GlcNAc transferase I-independent fucosylation pathway. Glycobiology (2006) 1.18
Glucosylceramide modulates membrane traffic along the endocytic pathway. J Lipid Res (2002) 1.17
A new class of anticancer alkylphospholipids uses lipid rafts as membrane gateways to induce apoptosis in lymphoma cells. Mol Cancer Ther (2007) 1.17
Function and glycosylation of plant-derived antiviral monoclonal antibody. Proc Natl Acad Sci U S A (2003) 1.16
The O-linked glycosylation of secretory/shed MUC1 from an advanced breast cancer patient's serum. Glycobiology (2008) 1.15
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. Mol Biol Cell (2005) 1.15
The carbohydrate epitope of the neutralizing anti-HIV-1 antibody 2G12. Adv Exp Med Biol (2003) 1.15
NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin. Ann Neurol (2004) 1.15