The fine structure of the cerebroside occurring in Gaucher's disease.
نویسنده
چکیده
Gaucher's disease is a metabolic disorder characterized by the accumulation of excessive quantities of glucocerebroside in the reticuloendothelial system. Brady et al. have demonstrated that individuals with this disease have deficient amounts of glucocerebrosidase, one of the enzymes required for complete degradation of the lipid of the red cell membrane.' The characteristic cell of Gaucher's disease has numerous membrane-enclosed sacs containing long slender tubules (Fig. 1). Cerebroside extracted with lipid solvents from the tissues of
منابع مشابه
Cerebroside Synthesis in Gaucher's Disease*
Familial lipodystrophic conditions, such as Gaucher's, Niemann-Pick, and Tay-Sachs disease, are characterized by the intracellular accumulation of abnormally large quantities of sphingolipids. In Gaucher's disease, the offending lipids are cerebrosides, while Niemann-Pick and Tay-Sachs diseases are characterized by the accumulation of sphingomyelin and gangliosides, respectively. In Gaucher's a...
متن کاملCerebroside synthesis in Gaucher's disease.
Familial lipodystrophic conditions, such as Gaucher's, Niemann-Pick, and Tay-Sachs disease, are characterized by the intracellular accumulation of abnormally large quantities of sphingolipids. In Gaucher's disease, the offending lipids are cerebrosides, while Niemann-Pick and Tay-Sachs diseases are characterized by the accumulation of sphingomyelin and gangliosides, respectively. In Gaucher's a...
متن کاملDemonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease.
The accumulation of abnormal quantities of glucocerebroside in the reticuloendothelial cells of patients with Gaucher's disease is well documented (2-7). Previous studies in this laboratory indicated no abnormality in cerebroside formation in spleen tissue obtained from patients with Gaucher's disease (8). These observations suggested that the biochemical lesion in these patients might be on th...
متن کاملThe lipoids of spleen and liver in various types of lipoidosis.
THE study of lipoid distribution in derangements of lipoid metabolism may be expected to throw new light on the physiology of lipoid formation, transport and storage, just as the knowledge of carbohydrate metabolism is based to a great extent on the study of diabetes. At least three pathological entities ofgeneralised "lipoid storage " disease or lipoidoses have been distinguished from clinical...
متن کاملSplenic lipids in Gaucher's disease.
Column chromatography (on cellulose, silicic acid, and Florisil) and thin-layer chromatography were employed for the separation and purification of lipid fractions from normal and Gaucher spleens. A new hydrolysis procedure, followed by paper chromatography, was used for identification of sugar moieties. A nonhydrolytic combined colorimetric procedure, with anthrone and orcinol, was used for th...
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ورودعنوان ژورنال:
- Proceedings of the National Academy of Sciences of the United States of America
دوره 61 2 شماره
صفحات -
تاریخ انتشار 1968