Pathways of sphingomyelin metabolism in cultured fibroblasts from normal and sphingomyelin lipidosis subjects.
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منابع مشابه
Pathways of sphingomyelin metabolism in cultured fibroblasts from normal and sphingomyelin lipidosis subjects.
The metabolism of endogenous sphingomyelin labeled with 32P or [methyl-3H]choline and of exogenous [choline-methyl-3H], [32P]-, or [N-acyl-1-14C]sphingomyelin was studied in normal and Niemann-Pick Type A (NP-A) cultured fibroblasts. Despite a greater than 96% decrease in lysosomal sphingomyelinase activity in the NP-A cells, they were able to degrade endogenously produced [32P]- or [methyl-3H]...
متن کاملThe role of sphingomyelin in phosphatidylcholine metabolism in cultured human fibroblasts from control and sphingomyelin lipidosis patients and in Chinese hamster ovary cells.
Human fibroblasts in culture take up exogenous [choline-Me-3H,32P]sphingomyelin (SM) from the medium and incorporate it into cellular SM and phosphatidylcholine [Spence, Clarke & Cook (1983) J. Biol. Chem. 258, 8595-8600]. The ratio of [3H]choline/[32P]Pi is similar in SM and phosphatidylcholine, indicating that the phosphocholine (P-Cho) moiety is transferred intact. Similar results are obtain...
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A 7-month-old Balinese cat with progressive neurological dysfunction had histopathological lesions of brain, liver, kidney, spleen, and lung consistent with a lysosomal storage disease. Ultrastructural examination revealed lysosomal hypertrophy with membranous inclusions. Hepatic sphingomyelin and cholesterol were elevated 10 times normal, and total phospholipids were increased 3.6 fold. Sphing...
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NAKASHIMA, M., KUDOH, T., SUKEGAWA, .K., MARUYAMA, K. and ORII, T. Metabolism of Sphingomyelin in Cultured Skin Fibroblasts from Patients with Different Types of Niemann-Pick Disease. Tohoku J. exp. Med., 1986, 148 (4), 365-371 The metabolism of [choline-methyl-14C] sphingomyelin in cultured skin fibroblasts from patients with different types of Niemann-Pick disease was measured 1 and 3 days af...
متن کاملImpairment of exogenous sphingomyelin degradation in cultured fibroblasts from familial hypercholesterolemia.
The degradation of exogenous sphingomyelin was investigated in cultured fibroblasts from normal subjects and subjects with familial hypercholesterolemia, either in whole medium or in lipoprotein-deficient medium. When introduced in whole medium, sphingomyelin degradation was significantly decreased (about 1.5-fold) in heterozygotes, and dramatically (about 4-fold) in homozygotes from familial h...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1983
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)32097-0