361 results on '"Boot, Rolf G."'
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2. Selective labelling of GBA2 in cells with fluorescent β-D-arabinofuranosyl cyclitol aziridines.
3. The development of a broad-spectrum retaining β-exo-galactosidase activity-based probe.
4. Corrigendum: Xylose‐Configured Cyclophellitols as Selective Inhibitors for Glucocerebrosidase
5. Mass spectrometric quantification of glucosylsphingosine in plasma and urine of type 1 Gaucher patients using an isotope standard
6. Corrigendum: Xylose‐Configured Cyclophellitols as Selective Inhibitors for Glucocerebrosidase
7. Glycosphingolipids and Insulin Resistance
8. Design and synthesis of 4′-O-alkyl-chitobiosyl-4-methylumbelliferone as human chitinase fluorogenic substrates
9. Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish
10. Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish
11. Phenotype diversity in type 1 Gaucher disease: discovering the genetic basis of Gaucher disease/hematologic malignancy phenotype by individual genome analysis
12. HEPES‐buffering of bicarbonate‐containing culture medium perturbs lysosomal glucocerebrosidase activity
13. Elevated Globotriaosylsphingosine Is a Hallmark of Fabry Disease
14. The cytosolic β-glucosidase GBA3 does not influence type 1 Gaucher disease manifestation
15. Increased YKL-40 and Chitotriosidase in Asthma and Chronic Obstructive Pulmonary Disease
16. Plasma chitotriosidase and CCL18 as surrogate markers for granulomatous macrophages in sarcoidosis
17. Xylose‐Configured Cyclophellitols as Selective Inhibitors for Glucocerebrosidase
18. Cell surface associated glycohydrolases in normal and Gaucher disease fibroblasts
19. Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies
20. CCL18: A urinary marker of Gaucher cell burden in Gaucher patients
21. Substrate reduction therapy of glycosphingolipid storage disorders
22. Evolution of mammalian chitinase(-like) members of family 18 glycosyl hydrolases
23. Fabry Disease: Molecular Basis, Pathophysiology, Diagnostics and Potential Therapeutic Directions
24. Human glucocerebrosidase mediates formation of xylosyl-cholesterol by β-xylosidase and transxylosidase reactions
25. Lyso-glycosphingolipids: presence and consequences
26. Manno-epi-cyclophellitols Enable Activity-Based Protein Profiling of Human α-Mannosidases and Discovery of New Golgi Mannosidase II Inhibitors
27. The Biology of the Gaucher Cell: The Cradle of Human Chitinases
28. Modulation of glycosphingolipid metabolism significantly improves hepatic insulin sensitivity and reverses hepatic steatosis in mice#
29. Common G102S polymorphism in chitotriosidase differentially affects activity towards 4-methylumbelliferyl substrates
30. Gaucher disease: a model disorder for biomarker discovery
31. Reduction of glycosphingolipid biosynthesis stimulates biliary lipid secretion in mice#
32. Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease
33. Biomarkers for lysosomal storage disorders: identification and application as exemplified by chitotriosidase in Gaucher disease
34. Myelin-laden macrophages are anti-inflammatory, consistent with foam cells in multiple sclerosis
35. Characterization of human phagocyte-derived chitotriosidase, a component of innate immunity
36. The chitinase-like protein YKL-40: A possible biomarker of inflammation and airway remodeling in severe pediatric asthma
37. Marked elevation of the chemokine CCL18/PARC in Gaucher disease: a novel surrogate marker for assessing therapeutic intervention
38. Chitotriosidase is the primary active chitinase in the human lung and is modulated by genotype and smoking habit
39. Role of μ-glucosidase 2 in aberrant glycosphingolipid metabolism: model of glucocerebrosidase deficiency in zebrafish
40. Functionalized Cyclophellitols Are Selective Glucocerebrosidase Inhibitors and Induce a Bona Fide Neuropathic Gaucher Model in Zebrafish
41. In vitro and in vivo comparative and competitive activity-based protein profiling of GH29 α-l-fucosidases† †Electronic supplementary information (ESI) available: Experimental part including synthesis procedures, characterization data, copies of NMR spectra of new compounds, supporting biological figures and crystallographic information. See DOI: 10.1039/c4sc03739a
42. A Fluorescence Polarization Activity-Based Protein Profiling Assay in the Discovery of Potent, Selective Inhibitors for Human Nonlysosomal Glucosylceramidase
43. Chemical Proteomic Analysis of Serine Hydrolase Activity in Niemann-Pick Type C Mouse Brain
44. Human Alpha Galactosidases Transiently Produced in Nicotiana benthamiana Leaves: New Insights in Substrate Specificities with Relevance for Fabry Disease
45. A Fluorescence Polarization Activity-Based Protein Profiling Assay in the Discovery of Potent, Selective Inhibitors for Human Nonlysosomal Glucosylceramidase
46. Beyond the Primary Biochemical Defect in Type 1 Gaucher Disease
47. Lysosomal Storage Diseases. For Better or Worse: Adapting to Defective Lysosomal Glycosphingolipid Breakdown
48. Human Alpha Galactosidases Transiently Produced in Nicotiana benthamiana Leaves: New Insights in Substrate Specificities with Relevance for Fabry Disease
49. Stabilization of Glucocerebrosidase by Active Site Occupancy
50. Correction to “Detection of Active Mammalian GH31 α-Glucosidases in Health and Disease Using In-Class, Broad-Spectrum Activity-Based Probes”
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