Sphingolipids, Sphingolipidoses and Allied Disorders: Proceedings of the Symposium on Sphingolipidoses and Allied Disorders held in Brooklyn, New York, October 25–27, 1971: Advances in Experimental Medicine and Biology, cartea 19
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Specificații
ISBN-13: 9781475765724
ISBN-10: 147576572X
Pagini: 720
Ilustrații: XXVII, 691 p. 154 illus.
Dimensiuni: 178 x 254 x 38 mm
Greutate: 1.23 kg
Ediția:1972
Editura: Springer Us
Colecția Springer
Seria Advances in Experimental Medicine and Biology
Locul publicării:New York, NY, United States
ISBN-10: 147576572X
Pagini: 720
Ilustrații: XXVII, 691 p. 154 illus.
Dimensiuni: 178 x 254 x 38 mm
Greutate: 1.23 kg
Ediția:1972
Editura: Springer Us
Colecția Springer
Seria Advances in Experimental Medicine and Biology
Locul publicării:New York, NY, United States
Public țintă
ResearchCuprins
Fine Structure of Early Tay-Sachs Disease.- Biopsy Diagnosis of Lipidoses: Background Considerations, General Concepts and Practical Aspects.- Lysosomal Diseases and Fibroblast Cultures: Biochemical and Electron Microscopic Observations.- The Morphogenesis and Biochemical Characteristics of Ceroid Isolated from Cases of Neuronal Ceroid-Lipofuscinosis.- Effect of Conditions of Extraction on the Extractability of Brain Gangliosides.- Gangliosides of CSF and Plasma: Their Relation to the Nervous System.- Ganglioside Inner Esters.- Human Brain Lipid Composition Changes With Age and Alterations in Some Pathological States: A New Method of Graphic Analysis.- Brain Glycoproteins and Inter-Cell Recognition: Tay-Sachs Disease and Intraneuronal Recognition.- A Fast Moving Protein in Tay-Sachs Disease.- Effect of Amino Acid Imbalance on Profiles and Protein Synthesis in Fetal Cerebral Cortex.- Purification and Properties of Two Sphingolipid Hydrolases.- Deficiency of Specific Proteins in the Inborn Errors of Mucopolysaccharide Metabolism.- The Degradation of Acid Mucopolysaccharides and the Mucopolysaccharidoses.- The Mucopolysaccharidoses as Lysosomal Diseases.- Recent Observations on Gaucher’s Disease.- Interaction of Enzymes with Lipid Substrates.- Problems in Prenatal Diagnosis Using Sphingolipid Hydrolase Assays.- Radioactive Precursor Incorporation Into Lipids of Humans with Cerebral Lipidoses: 1-14C-Glucosamine, U3H-Serine, and 3H-Acetate.- An Investigation of the Metabolism of Tay-Sachs Ganglioside Specifically Labeled in Critical Portions of the Molecule.- Chemistry and Metabolism of Glycosphingolipids in Fabry’s Disease.- The Chemical Pathology of Tay-Sachs Disease.- Sandhoff’s Disease: Studies on the Enzyme Defect in Homozygotes and Detection of Heterozygotes.- AnUnusual Case of GM2-Gangliosidosis With Deficiency of Hexosaminidase A and B.- Sandhoff’s Disease: Ultrastructural and Biochemical Studies.- Biochemical Studies on GM1-Gangliosidosis and Ceramide Trihexosidosis.- Chemical Pathology of Tay-Sachs Disease in the Fetus.- Glycosphingolipid Abnormalities in Liver From Patients with Glycosphingolipid and Mucopolysaccharide Storage Diseases.- Recent Advances in Metachromatic Leukodystrophy.- Liver Glycolipids, Steroid Sulfates and Steroid Sulfatases in a Form of Metachromatic Leukodystrophy Associated with Multiple Sulfatase Deficiencies.- In Vitro Studies in Sulfatide Lipidosis.- Factors Affecting the Metabolism of Galactocerebroside and Glucocerebroside.- Further Studies on Galactocerebroside ?-Galacto sidase in Globoid Cell Leukodystrophy.- Brain Gangliosides in Krabbe Disease.- Phytanic Acid Storage Disease.- Studies on a Case of Lipogranulomatosis (Farber’s Disease) With Protracted Course.- Biochemical Studies on Brain Explants and Fibroblast Cultures in Batten’s Disease.- Population Dynamics of Tay-Sachs Disease. II. What Confers the Selective Advantage Upon the Jewish Heterozygote?.- Effect of Maternal Protein Deficiency on Ganglioside Metabolism in Neonatal Rat Brain.- Sphingolipidoses: Detection of Heterozygotes and Homozygotes.- Pre- and Postnatal Detection of Tay-Sachs Disease. A Comparative Study of Biochemical Screening Methods.- Heterozygote Detection in Tay-Sachs Disease: A Prototype Community Screening Program for the Prevention of Recessive Genetic Disorders.- Metabolic Changes Following Splenic Transplantation in a Case of Gaucher’s Disease.- Studies on the Metabolic Control of Fabry’s Disease Through Kidney Transplantation.- The Induction of Sulfatide, Ganglioside and Cerebroside Storage inOrganized Nervous System Cultures.- Nutritional Support, Including Intravenous Alimentation, for the Infant with Wolman’s Disease.- Precocious Puberty in Tay-Sachs Disease.- Summary Remarks.