Published in Nat Rev Neurosci on September 01, 2005
Autophagy failure in Alzheimer's disease--locating the primary defect. Neurobiol Dis (2011) 2.14
Common and uncommon pathogenic cascades in lysosomal storage diseases. J Biol Chem (2010) 1.85
Midbody ring disposal by autophagy is a post-abscission event of cytokinesis. Nat Cell Biol (2008) 1.70
Neuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCL. Nat Neurosci (2013) 1.58
Nanomedicine in the diagnosis and therapy of neurodegenerative disorders. Prog Polym Sci (2007) 1.41
Autophagy in neurodegeneration and development. Biochim Biophys Acta (2008) 1.38
Clues to neuro-degeneration in Niemann-Pick type C disease from global gene expression profiling. PLoS One (2006) 1.37
Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models. Mol Genet Metab (2010) 1.35
Cell replacement therapy in neurological disease. Philos Trans R Soc Lond B Biol Sci (2006) 1.29
Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C. Hum Mol Genet (2012) 1.26
Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts. Pathogenetics (2008) 1.20
The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts. Mol Ther (2009) 1.17
Substrate reduction therapy of glycosphingolipid storage disorders. J Inherit Metab Dis (2006) 1.16
Lysosomal storage diseases--the horizon expands. Nat Rev Neurol (2013) 1.15
The novel endosomal membrane protein Ema interacts with the class C Vps-HOPS complex to promote endosomal maturation. J Cell Biol (2010) 1.13
Survival advantage of neonatal CNS gene transfer for late infantile neuronal ceroid lipofuscinosis. Exp Neurol (2008) 1.13
Pathophysiology of neuropathic lysosomal storage disorders. J Inherit Metab Dis (2010) 1.13
Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy. Brain Res (2009) 1.12
Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo. PLoS Genet (2010) 1.12
Screening for pharmacological chaperones in Fabry disease. Biochem Biophys Res Commun (2007) 1.11
Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy. J Inherit Metab Dis (2010) 1.10
Macroautophagy is not directly involved in the metabolism of amyloid precursor protein. J Biol Chem (2010) 1.03
Progenitor cell-based treatment of the pediatric myelin disorders. Arch Neurol (2011) 1.03
Enzyme, cell and gene-based therapies for metachromatic leukodystrophy. J Inherit Metab Dis (2007) 0.99
Astrocyte dysfunction triggers neurodegeneration in a lysosomal storage disorder. Proc Natl Acad Sci U S A (2012) 0.98
The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol. Hum Mol Genet (2012) 0.97
Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice. Hum Mol Genet (2014) 0.94
The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis. Nat Chem Biol (2014) 0.93
Role of endolysosomes in HIV-1 Tat-induced neurotoxicity. ASN Neuro (2012) 0.92
Murine muscle cell models for Pompe disease and their use in studying therapeutic approaches. Mol Genet Metab (2009) 0.92
Differential regulation of sphingomyelin synthesis and catabolism in oligodendrocytes and neurons. J Neurochem (2008) 0.91
Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain. J Lipid Res (2011) 0.90
Lysosomal adaptation: how the lysosome responds to external cues. Cold Spring Harb Perspect Biol (2014) 0.90
Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis. J Neuropathol Exp Neurol (2011) 0.89
Strategies for delivery of therapeutics into the central nervous system for treatment of lysosomal storage disorders. Drug Deliv Transl Res (2012) 0.89
Promising CNS-directed enzyme replacement therapy for lysosomal storage diseases. Exp Neurol (2009) 0.88
Development of a Rab9 transgenic mouse and its ability to increase the lifespan of a murine model of Niemann-Pick type C disease. Am J Pathol (2008) 0.88
Lysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration. Cell (2016) 0.87
Neural stem cell transplantation benefits a monogenic neurometabolic disorder during the symptomatic phase of disease. Stem Cells (2009) 0.86
The sensitivity of murine spermiogenesis to miglustat is a quantitative trait: a pharmacogenetic study. Reprod Biol Endocrinol (2007) 0.85
Deletion in the N-terminal half of olfactomedin 1 modifies its interaction with synaptic proteins and causes brain dystrophy and abnormal behavior in mice. Exp Neurol (2013) 0.82
Update on treatment of lysosomal storage diseases. Acta Myol (2007) 0.81
Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation. J Neuroinflammation (2012) 0.81
Clinical aspects of neuropathic lysosomal storage disorders. J Inherit Metab Dis (2010) 0.81
Transplantation and magnetic resonance imaging of canine neural progenitor cell grafts in the postnatal dog brain. J Neuropathol Exp Neurol (2008) 0.80
Early changes in the apparent diffusion coefficient (ADC) in a mouse model of Sandhoff's disease occur prior to disease symptoms and behavioral deficits. Magn Reson Med (2009) 0.79
Mammalian sialyltransferase ST3Gal-II: its exchange sialylation catalytic properties allow labeling of sialyl residues in mucin-type sialylated glycoproteins and specific gangliosides. Biochemistry (2011) 0.78
Inherited and acquired disorders of myelin: The underlying myelin pathology. Exp Neurol (2016) 0.77
Design and synthesis of versatile ganglioside probes for carbohydrate microarrays. Glycoconj J (2008) 0.77
Structures of mammalian ER α-glucosidase II capture the binding modes of broad-spectrum iminosugar antivirals. Proc Natl Acad Sci U S A (2016) 0.77
Bis(monoacylglycero)phosphate and ganglioside GM1 spontaneously form small homogeneous vesicles at specific concentrations. Biochem Biophys Res Commun (2010) 0.76
Invertebrate models of lysosomal storage disease: what have we learned so far? Invert Neurosci (2011) 0.76
Glial progenitor cell-based treatment of the childhood leukodystrophies. Exp Neurol (2016) 0.75
A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline. Neurobiol Dis (2016) 0.75
BK channel agonist represents a potential therapeutic approach for lysosomal storage diseases. Sci Rep (2016) 0.75
Differential sensitivity of mouse strains to an N-alkylated imino sugar: glycosphingolipid metabolism and acrosome formation. Pharmacogenomics (2008) 0.75
The effects of early and late bone marrow transplantation in siblings with alpha-mannosidosis. Is early haematopoietic cell transplantation the preferred treatment option? J Inherit Metab Dis (2010) 0.75
Clinical or ATPase domain mutations in ABCD4 disrupt the interaction between the vitamin B12-trafficking proteins ABCD4 and LMBD1. J Biol Chem (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
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
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
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
Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone. J Cell Mol Med (2008) 1.33
4-C-Me-DAB and 4-C-Me-LAB - enantiomeric alkyl-branched pyrrolidine iminosugars - are specific and potent α-glucosidase inhibitors; acetone as the sole protecting group. Tetrahedron Lett (2011) 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
Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport. J Biol Chem (2004) 1.22
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
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
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 carbohydrate epitope of the neutralizing anti-HIV-1 antibody 2G12. Adv Exp Med Biol (2003) 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
NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin. Ann Neurol (2004) 1.15