800 results on '"Sandhoff, K."'
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2. Glycosphingolipids and sphingolipid activator proteins: Cell biology, biochemistry and molecular genetics
3. Topology and Regulation of Ganglioside Metabolism — Function and Pathobiochemistry of Sphingolipid Activator Proteins
4. Correlation between Residual Activity of a Lysosomal Hydrolase and the Severity of the Resulting Storage Disease
5. Glycolipids — Intracellular Movement and Storage Diseases
6. AB Variant of Infantile G M2 Gangliosidosis: Deficiency of a Factor Necessary for Stimulation of Hexosaminidase A-Catalyzed Degradation of Ganglioside G M2 and Glycolipid G A2
7. Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study
8. Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease
9. Restoration of the GM2 Ganglioside Metabolism in Bone Marrow–Derived Stromal Cells from Tay-Sachs Disease Animal Model
10. Uptake of Mannose-Terminal Glucocerebrosidase in Cultured Human Cholinergic and Dopaminergic Neuron Cell Lines
11. A non-glycosylated and functionally deficient mutant (N215H) of the sphingolipid activator protein B (SAP-B) in a novel case of metachromatic leukodystrophy (MLD)
12. Gangliosides are Transported from the Plasma Membrane to Intralysosomal Membranes as Revealed by Immuno-Electron Microscopy
13. Glycosphingolipid degradation and animal models of GM2-gangliosidoses
14. Dysregulation of channel activating protease 1 in the epidermis of the mouse: 34
15. FT-IR-ATR Spectroscopy of Oriented Phospholipid-Ganglioside Bilayers
16. A direct gene transfer strategy via brain internal capsule reverses the biochemical defect in Tay–Sachs disease
17. [16] Purification and characterization of recombinant human acid sphingomyelinase expressed in insect Sf21 cells
18. [23] Purification of acid ceramidase from human placenta
19. Metabolism of GM1 ganglioside in cultured skin fibroblasts: anomalies in gangliosidoses, sialidoses, and sphingolipid activator protein (SAP, saposin) 1 and prosaposin deficient disorders
20. Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease
21. Glycosphingolipid metabolism at membrane–water interphases and its pathobiochemistry
22. Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease
23. Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3
24. SUSPECTED MILD ADULT SAP-B DEFICIENCY
25. Bone marrow transplantation in metachromatic leukodystrophy caused by saposin-B deficiency: A case report with a 3-year follow-up period
26. Sphingolipid Metabolism: Sphingoid Analogs, Sphingolipid Activator Proteins, and the Pathology of the Cella
27. [52] Human acid sphingomyelinase from human urine
28. CIS-4-METHYLSPHINGOSINE-l-PHOSPHATE AS A POTENTIAL METABOLICALLY STABLE MIMETIC OF SPHINGOSINE-l-PHOSPHATE ACTIVITY
29. PROCESSING OF SPHINGOLIPID ACTIVATOR PROTEINS AND THE TOPOLOGY OF LYSOSOMAL DIGESTION
30. CIS-4-METHYLSPHINGOSINE-1-PHOSPHATE AS A POTENTIAL METABOLICALLY STABLE MIMETIC OF SPHINGOSINE-1-PHOSPHATE ACTIVITY
31. MICE TOTALLY DEFICIENT IN β-HEXOSAMINIDASE α AND β SUBUNITS SHOW AN ADDITIONAL MUCOPOLYSACCHARIDOSIS PHENOTYPE
32. GENETIC SPHINGOLIPID ACTIVATOR PROTEIN(SAP) DEFICIENCIES IN HUMAN AND A "KNOCKOUT" MOUSE
33. Glycosphingolipid Activator Proteins
34. Immunological Studies on Lysosomal Sphingomyelinase: Immunization Procedures, Properties of Polyclonal and Monoclonal Antibodies Obtained and Effect of Triton X-100 on Binding of Enzyme Activity
35. Purification and Properties of Acid Sphingomyelinase from Human Urine
36. Gangliosides: Uptake, Intracellular Transport and Metabolism in Normal and Mutant Cells
37. Acid Sphingomyelinase from Human Urine: Purification and Characterisation
38. Substrate Specificity of Hexosaminidase a Isolated from the Liver of a Patient with a Rare Form (AB Variant) of Infantile GM2 Gangliosidosis and Control Tissues
39. Studies on Bovine Brain Membrane-Bound Neuraminidase (Sialidase)
40. Fundamentals of Ganglioside Catabolism
41. In Vitro Incorporation and Metabolism of Gangliosides
42. Concepts of Ganglioside Metabolism
43. Hexosaminidase I Indicates Maturation Disarrangement in Acute Leukemias
44. Biochemistry of Gangliosidoses
45. S7.1 Topology and regulation of glycolipid metabolism
46. Glycolipids of the cell surface: Biosynthesis and topology of metabolism
47. Lipid-protein interactions and effect of local anesthetics in acetylcholine receptor-rich membranes from Torpedo marmorata electric organ
48. Construction and Characterization of Acid Ceramidase Deficient Mice: A New Model of Farber Disease
49. Genetic complementation in somatic cell hybrids of four variants of infantile GM2 gangliosidosis
50. Substrate specificities of a bacterial sialidase and rat liver ganglioside GM3 sialyltransferase
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