Published in J Org Chem on January 23, 2007
Evaluation of quinazoline analogues as glucocerebrosidase inhibitors with chaperone activity. J Med Chem (2011) 1.05
β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology. J Biol Chem (2013) 0.97
Adamantane-1-thio-amide. Acta Crystallogr Sect E Struct Rep Online (2009) 0.92
N4-phenyl modifications of N2-(2-hydroxyl)ethyl-6-(pyrrolidin-1-yl)-1,3,5-triazine-2,4-diamines enhance glucocerebrosidase inhibition by small molecules with potential as chemical chaperones for Gaucher disease. Bioorg Med Chem Lett (2007) 0.89
Synthesis and evaluation of eight- and four-membered iminosugar analogues as inhibitors of testicular ceramide-specific glucosyltransferase, testicular β-glucosidase 2, and other glycosidases. J Org Chem (2012) 0.88
Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease. PLoS One (2016) 0.82
Synthesis of lipophilic 1-deoxygalactonojirimycin derivatives as D-galactosidase inhibitors. Beilstein J Org Chem (2010) 0.81
Identification of potent and selective glucosylceramide synthase inhibitors from a library of N-alkylated iminosugars. ACS Med Chem Lett (2010) 0.80
Development of targeted therapies for Parkinson's disease and related synucleinopathies. J Lipid Res (2014) 0.80
1-Deoxynojirimycins with dansyl capped N-substituents as probes for Morbus Gaucher affected cell lines. Carbohydr Res (2010) 0.80
Geometric attributes of retaining glycosyltransferase enzymes favor an orthogonal mechanism. PLoS One (2013) 0.79
Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice. PLoS One (2015) 0.78
Correction of liver steatosis by a hydrophobic iminosugar modulating glycosphingolipids metabolism. PLoS One (2012) 0.78
A sensitive gel-based method combining distinct cyclophellitol-based probes for the identification of acid/base residues in human retaining β-glucosidases. J Biol Chem (2014) 0.78
Preliminary joint neutron time-of-flight and X-ray crystallographic study of human ABO(H) blood group A glycosyltransferase. Acta Crystallogr Sect F Struct Biol Cryst Commun (2011) 0.78
Fluorous iminoalditols: a new family of glycosidase inhibitors and pharmacological chaperones. Chembiochem (2010) 0.77
1-Deoxy-D-galactonojirimycins with dansyl capped N-substituents as β-galactosidase inhibitors and potential probes for GM1 gangliosidosis affected cell lines. Carbohydr Res (2011) 0.75
Elevated globotriaosylsphingosine is a hallmark of Fabry disease. Proc Natl Acad Sci U S A (2008) 2.87
Treatment of Fabry disease with different dosing regimens of agalsidase: effects on antibody formation and GL-3. Mol Genet Metab (2008) 2.60
Asymmetric proteasome segregation as a mechanism for unequal partitioning of the transcription factor T-bet during T lymphocyte division. Immunity (2011) 2.34
Proteasome inhibitors: an expanding army attacking a unique target. Chem Biol (2012) 2.26
Enzyme therapy for Fabry disease: neutralizing antibodies toward agalsidase alpha and beta. Kidney Int (2004) 1.89
Specificity and affinity of natural product cyclopentapeptide inhibitors against A. fumigatus, human, and bacterial chitinases. Chem Biol (2005) 1.80
Characterization of human phagocyte-derived chitotriosidase, a component of innate immunity. Int Immunol (2005) 1.76
Evolution of mammalian chitinase(-like) members of family 18 glycosyl hydrolases. Genetics (2007) 1.73
Structure of human chitotriosidase. Implications for specific inhibitor design and function of mammalian chitinase-like lectins. J Biol Chem (2002) 1.71
Analysis of protease activity in live antigen-presenting cells shows regulation of the phagosomal proteolytic contents during dendritic cell activation. J Exp Med (2002) 1.64
A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease. Mol Genet Metab (2007) 1.61
Thioglycosides in sequential glycosylation strategies. Chem Soc Rev (2005) 1.60
A fluorescent broad-spectrum proteasome inhibitor for labeling proteasomes in vitro and in vivo. Chem Biol (2006) 1.54
Force majeure: therapeutic measures in response to restricted supply of imiglucerase (Cerezyme) for patients with Gaucher disease. Blood Cells Mol Dis (2009) 1.54
Treatment of Fabry disease: outcome of a comparative trial with agalsidase alfa or beta at a dose of 0.2 mg/kg. PLoS One (2007) 1.54
Identification of the non-lysosomal glucosylceramidase as beta-glucosidase 2. J Biol Chem (2006) 1.52
Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease. Int J Biochem Cell Biol (2005) 1.50
Assessing the statistical validity of proteomics based biomarkers. Anal Chim Acta (2007) 1.49
Selective inhibitor of proteasome's caspase-like sites sensitizes cells to specific inhibition of chymotrypsin-like sites. Chem Biol (2009) 1.47
The caspase-like sites of proteasomes, their substrate specificity, new inhibitors and substrates, and allosteric interactions with the trypsin-like sites. J Biol Chem (2003) 1.46
Synthesis of pH-activatable red fluorescent BODIPY dyes with distinct functionalities. Org Lett (2011) 1.44
Marked elevation of the chemokine CCL18/PARC in Gaucher disease: a novel surrogate marker for assessing therapeutic intervention. Blood (2003) 1.44
Ph2SO/Tf2O: a powerful promotor system in chemoselective glycosylations using thioglycosides. Org Lett (2003) 1.42
Glycosphingolipids--nature, function, and pharmacological modulation. Angew Chem Int Ed Engl (2009) 1.41
Marked differences in tissue-specific expression of chitinases in mouse and man. J Histochem Cytochem (2005) 1.29
Muscle wasting in aged, sarcopenic rats is associated with enhanced activity of the ubiquitin proteasome pathway. J Biol Chem (2010) 1.28
Ultrasensitive in situ visualization of active glucocerebrosidase molecules. Nat Chem Biol (2010) 1.26
Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients. Orphanet J Rare Dis (2011) 1.25
Chemistry in living cells: detection of active proteasomes by a two-step labeling strategy. Angew Chem Int Ed Engl (2003) 1.25
Distinct uptake mechanisms but similar intracellular processing of two different toll-like receptor ligand-peptide conjugates in dendritic cells. J Biol Chem (2007) 1.23
Glycosphingolipids and insulin resistance. Prog Lipid Res (2009) 1.23
Quantification of skeletal involvement in adults with type I Gaucher's disease: fat fraction measured by Dixon quantitative chemical shift imaging as a valid parameter. AJR Am J Roentgenol (2002) 1.23
Crystal structure of HslUV complexed with a vinyl sulfone inhibitor: corroboration of a proposed mechanism of allosteric activation of HslV by HslU. J Mol Biol (2002) 1.21
Analysis and quantification of diagnostic serum markers and protein signatures for Gaucher disease. Mol Cell Proteomics (2007) 1.21
A modular strategy toward the synthesis of heparin-like oligosaccharides using monomeric building blocks in a sequential glycosylation strategy. J Am Chem Soc (2005) 1.17
Transglycosidase activity of chitotriosidase: improved enzymatic assay for the human macrophage chitinase. J Biol Chem (2003) 1.16
Structure and reactivity of an asymmetric complex between HslV and I-domain deleted HslU, a prokaryotic homolog of the eukaryotic proteasome. J Mol Biol (2003) 1.13
Specific cell-permeable inhibitor of proteasome trypsin-like sites selectively sensitizes myeloma cells to bortezomib and carfilzomib. Chem Biol (2011) 1.13
Thioglycuronides: synthesis and application in the assembly of acidic oligosaccharides. Org Lett (2004) 1.11
Limitations of drug registries to evaluate orphan medicinal products for the treatment of lysosomal storage disorders. Orphanet J Rare Dis (2011) 1.11
Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease. Blood Cells Mol Dis (2005) 1.11
Modulation of glycosphingolipid metabolism significantly improves hepatic insulin sensitivity and reverses hepatic steatosis in mice. Hepatology (2009) 1.09
Convection perfusion of glucocerebrosidase for neuronopathic Gaucher's disease. Ann Neurol (2005) 1.08
Reducing glycosphingolipid content in adipose tissue of obese mice restores insulin sensitivity, adipogenesis and reduces inflammation. PLoS One (2009) 1.08
Bioorthogonal organic chemistry in living cells: novel strategies for labeling biomolecules. Org Biomol Chem (2004) 1.08
Mixing of peptides and electrophilic traps gives rise to potent, broad-spectrum proteasome inhibitors. Org Biomol Chem (2007) 1.07
HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma. Clin Chem (2007) 1.07
Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies. J Inherit Metab Dis (2011) 1.07
Small-molecule inhibitors and probes for ubiquitin- and ubiquitin-like-specific proteases. Chembiochem (2005) 1.07
Monitoring of Gaucher patients with a novel chitotriosidase assay. Clin Chim Acta (2007) 1.05
Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome. PLoS One (2012) 1.04
The use of cyclic bifunctional protecting groups in oligosaccharide synthesis--an overview. Carbohydr Res (2006) 1.04
Pro-inflammatory delipidizing cytokines reduce adiponectin secretion from human adipocytes without affecting adiponectin oligomerization. J Endocrinol (2007) 1.03
Automated solid-phase synthesis of β-mannuronic acid alginates. Angew Chem Int Ed Engl (2012) 1.02
Recombinant enzyme therapy for Fabry disease: absence of editing of human alpha-galactosidase A mRNA. Am J Hum Genet (2002) 1.02
Paradoxical increase in TAG and DAG content parallel the insulin sensitizing effect of unilateral DGAT1 overexpression in rat skeletal muscle. PLoS One (2011) 1.01
Activity-based profiling reveals reactivity of the murine thymoproteasome-specific subunit beta5t. Chem Biol (2010) 1.01
Development of an activity-based probe and in silico design reveal highly selective inhibitors for diacylglycerol lipase-α in brain. Angew Chem Int Ed Engl (2013) 1.01
Functional proteomics of the active cysteine protease content in Drosophila S2 cells. Mol Cell Proteomics (2003) 1.01
The cytosolic β-glucosidase GBA3 does not influence type 1 Gaucher disease manifestation. Blood Cells Mol Dis (2010) 0.99
Crystal structures of allosamidin derivatives in complex with human macrophage chitinase. J Biol Chem (2003) 0.99
Quantification of globotriaosylsphingosine in plasma and urine of fabry patients by stable isotope ultraperformance liquid chromatography-tandem mass spectrometry. Clin Chem (2012) 0.98
Stereodirecting effect of the pyranosyl C-5 substituent in glycosylation reactions. J Org Chem (2009) 0.97
Syringolin A selectively labels the 20 S proteasome in murine EL4 and wild-type and bortezomib-adapted leukaemic cell lines. Chembiochem (2009) 0.97
A cell-permeable inhibitor and activity-based probe for the caspase-like activity of the proteasome. Bioorg Med Chem Lett (2007) 0.96
The impact of oxacarbenium ion conformers on the stereochemical outcome of glycosylations. Carbohydr Res (2010) 0.96
Relative quantification of proteasome activity by activity-based protein profiling and LC-MS/MS. Nat Protoc (2013) 0.96
Screening for Fabry disease using whole blood spots fails to identify one-third of female carriers. Clin Chim Acta (2005) 0.96
Sequential one-pot glycosylations using 1-hydroxyl and 1-thiodonors. Org Lett (2003) 0.94
Acetylene functionalized BODIPY dyes and their application in the synthesis of activity based proteasome probes. Bioorg Med Chem Lett (2007) 0.94
Nature of pharmacophore influences active site specificity of proteasome inhibitors. J Biol Chem (2010) 0.94
Human B lymphoblastoid cells contain distinct patterns of cathepsin activity in endocytic compartments and regulate MHC class II transport in a cathepsin S-independent manner. J Leukoc Biol (2004) 0.94
Small fiber neuropathy in Fabry disease. Mol Genet Metab (2012) 0.93
Conjugation of nucleosides and oligonucleotides by [3+2] cycloaddition. J Org Chem (2007) 0.93
Automated solid-phase synthesis of hyaluronan oligosaccharides. Org Lett (2012) 0.93
alpha-Galactosidase A deficiency in Dutch patients on dialysis: a critical appraisal of screening for Fabry disease. Nephrol Dial Transplant (2003) 0.93