Sphingolipids, Sphingolipidoses and Allied Disorders

Table of Contents

1. Altmetric Badge
   Book Overview
2. Altmetric Badge
   Chapter 1 Fine Structure of Early Tay-Sachs Disease
3. Altmetric Badge
   Chapter 2 Biopsy Diagnosis of Lipidoses: Background Considerations, General Concepts and Practical Aspects
4. Altmetric Badge
   Chapter 3 Lysosomal Diseases and Fibroblast Cultures: Biochemical and Electron Microscopic Observations
5. Altmetric Badge
   Chapter 4 The Morphogenesis and Biochemical Characteristics of Ceroid Isolated from Cases of Neuronal Ceroid-Lipofuscinosis
6. Altmetric Badge
   Chapter 5 Effect of Conditions of Extraction on the Extractability of Brain Gangliosides
7. Altmetric Badge
   Chapter 6 Gangliosides of CSF and Plasma: Their Relation to the Nervous System
8. Altmetric Badge
   Chapter 7 Ganglioside Inner Esters
9. Altmetric Badge
   Chapter 8 Human Brain Lipid Composition Changes with Age and Alterations in Some Pathological States: A New Method of Graphic Analysis
10. Altmetric Badge
    Chapter 9 Brain Glycoproteins and Inter-Cell Recognition: Tay-Sachs’ Disease and Intraneuronal Recognition
11. Altmetric Badge
    Chapter 10 A Fast Moving Protein in Tay-Sachs Disease
12. Altmetric Badge
    Chapter 11 Effect of Amino Acid Imbalance on Polyribosome Profiles and Protein Synthesis in Fetal Cerebral Cortex
13. Altmetric Badge
    Chapter 12 Purification and Properties of Two Sphingolipid Hydrolases
14. Altmetric Badge
    Chapter 13 Deficiency of Specific Proteins in the Inborn Errors of Mucopolysaccharide Metabolism
15. Altmetric Badge
    Chapter 14 The Degradation of Acid Mucopolysaccharides and the Mucopolysaccharidoses
16. Altmetric Badge
    Chapter 15 The Mucopolysaccharidoses as Lysosomal Diseases
17. Altmetric Badge
    Chapter 16 Recent Observations on Gaucher’s Disease
18. Altmetric Badge
    Chapter 17 Interaction of Enzymes with Lipid Substrates
19. Altmetric Badge
    Chapter 18 Problems in Prenatal Diagnosis Using Sphingolipid Hydrolase Assays
20. Altmetric Badge
    Chapter 19 Radioactive Precursor Incorporation into Lipids of Humans with Cerebral Lipidoses:1- 14 C-Glucosamine, U 3 H-Serine, and 3 H-Acetate
21. Altmetric Badge
    Chapter 20 An Investigation of the Metabolism of Tay-Sachs Ganglioside Specifically Labeled in Critical Portions of the Molecule
22. Altmetric Badge
    Chapter 21 Chemistry and Metabolism of Glycosphingolipids in Fabry’s Disease
23. Altmetric Badge
    Chapter 22 The Chemical Pathology of Tay-Sachs Disease
24. Altmetric Badge
    Chapter 23 Sandhoff’s Disease: Studies on the Enzyme Defect in Homozygotes and Detection of Heterozygotes
25. Altmetric Badge
    Chapter 24 An Unusual Case of G M2 -Gangliosidosis with Deficiency of Hexosaminidase A and B
26. Altmetric Badge
    Chapter 25 Sandhoff’s Disease: Ultrastructural and Biochemical Studies
27. Altmetric Badge
    Chapter 26 Biochemical Studies on G M1 -Gangliosidosis and Ceramide Trihexosidosis
28. Altmetric Badge
    Chapter 27 Chemical Pathology of Tay-Sachs Disease in the Fetus
29. Altmetric Badge
    Chapter 28 Glycosphingolipid Abnormalities in Liver from Patients with Glycosphingolipid and Mucopolysaccharide Storage Diseases
30. Altmetric Badge
    Chapter 29 Recent Advances in Metachromatic Leukodystrophy
31. Altmetric Badge
    Chapter 30 Liver Glycolipids, Steroid Sulfates and Steroid Sulfatases in a Form of Metachromatic Leukodystrophy Associated with Multiple Sulfatase Deficiencies
32. Altmetric Badge
    Chapter 31 In Vitro Studies in Sulfatide Lipidosis
33. Altmetric Badge
    Chapter 32 Factors Affecting the Metabolism of Galactocerebroside and Glucocerebroside
34. Altmetric Badge
    Chapter 33 Further Studies on Galactocerebroside β -Galactosidase in Globoid Cell Leukodystrophy
35. Altmetric Badge
    Chapter 34 Brain Gangliosides in Krabbe Disease
36. Altmetric Badge
    Chapter 35 Phytanic Acid Storage Disease
37. Altmetric Badge
    Chapter 36 Studies on a Case of Lipogranulomatosis (Farber’s Disease) with Protracted Course
38. Altmetric Badge
    Chapter 37 Biochemical Studies on Brain Explants and Fibroblast Cultures in Batten’s Disease
39. Altmetric Badge
    Chapter 38 Population Dynamics of Tay-Sachs Disease. II. What Confers the Selective Advantage Upon the Jewish Heterozygote?
40. Altmetric Badge
    Chapter 39 Effect of Maternal Protein Deficiency on Ganglioside Metabolism in Neonatal Rat Brain
41. Altmetric Badge
    Chapter 40 Sphingolipidoses: Detection of Heterozygotes and Homozygotes
42. Altmetric Badge
    Chapter 41 Pre- and Postnatal Detection of Tay-Sachs Disease. A Comparative Study of Biochemical Screening Methods
43. Altmetric Badge
    Chapter 42 Heterozygote Detection in Tay-Sachs Disease: A Prototype Community Screening Program for the Prevention of Recessive Genetic Disorders
44. Altmetric Badge
    Chapter 43 Metabolic Changes Following Splenic Transplantation in a Case of Gaucher’s Disease
45. Altmetric Badge
    Chapter 44 Studies on the Metabolic Control of Fabry’s Disease Through Kidney Transplantation
46. Altmetric Badge
    Chapter 45 The Induction of Sulfatide, Ganglioside and Cerebroside Storage in Organized Nervous System Cultures
47. Altmetric Badge
    Chapter 46 Nutritional Support, Including Intravenous Alimentation, for the Infant with Wolman’s Disease
48. Altmetric Badge
    Chapter 47 Precocious Puberty in Tay-Sachs Disease
49. Altmetric Badge
    Chapter 48 Summary Remarks