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Digestive Capacity of Lysosomes
Structure and Enzymic Degradation of Sphingolipids
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3-galactosidase A. B. Novikoff abnormal accumulation acid hydrolases acid phosphatase acid phosphatase activity Acta Amer amniotic fluid appears Arch assay autophagic Biochem Biophys Brady brain cathepsin Cell Biol ceramide cerebroside Chapter Chem chondroitin sulfate Clin clinical contain cultured cystine cystinosis cytoplasm defect deficiency degradation demonstrated dermatan sulfate diagnosis digestion Dingle disorders Duve electron etal Fabry's disease fatty acids fibroblasts fraction galactocerebroside galactose ganglioside Gaucher's disease genetic globoid cells glucocerebroside glycogen glycolipids glycosaminoglycans GMi-gangliosidosis granules heparan sulfate heterozygotes hexosaminidase Hoof Hurler's hydrolyzed inborn lysosomal diseases infantile intracellular keratan kidney leukocytes leukodystrophy lipid lysosomal enzymes macrophages material membrane metabolic metachromatic molecules mucopolysaccharide mucopolysaccharidosis myelin Neufeld neuronal Niemann-Pick's disease normal O'Brien observed Pathol Pathology patients Pediat plasma present Proc protein residual bodies serum sphingolipids sphingomyelin spleen stain storage diseases structure studies substrate sulfatase sulfatide Suzuki syndrome Tay-Sachs tissues ultrastructural urine vacuoles variant Wolman's disease