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Current issue subscribe archive rss & alerts contact loading article tools view pdf cite this article e-mail this article share this article send a letter information on reuse standard abbreviations author information find articles by tallman, j. F. In: jci | pubmed | google scholar find articles by brady, r. O. In: jci | pubmed | google scholar need help? E-mail the jci published in volume 51, issue 9 (september 1972) j clin invest. 1972;51(9):2339–2345. Doi:10. 1172/jci107045. Copyright â© 1972, the american society for clinical investigation. Articles the metabolism of tay-sachs ganglioside: catabolic studies with lysosomal enzymes from normal and tay-sachs brain tissue john f. Tallman, william g. Johnson and roscoe o. Brady developmental and metabolic neurology branch, national institute of neurological diseases and stroke, national institutes of health, bethesda, maryland 20014 department of biochemistry, georgetown university school of medicine, washington, d. C. 20007 published september 1972 the catabolism of tay-sachs ganglioside, n-acetylgalactosaminyl- (n-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from tay-sachs patients. Utilizing tay-sachs ganglioside labeled with 14c in the n-acetylgalactosaminyl portion or 3h in the n-acetylneuraminosyl portion, the catabolism of tay-sachs ganglioside may be initiated by either the removal of the molecule of n-acetylgalactosamine or n-acetylneuraminic acid. The activity of the n-acetylgalactosamine-cleaving enzyme (hexosaminidase) is drastically diminished in such preparations from tay-sachs brain whereas the activity of the n-acetylneuraminic acid-cleaving enzyme (neuraminidase) is at a normal level. Total hexosaminidase activity as measured with an artificial fluorogenic substrate is increased in tissues obtained from patients with the b variant form of tay-sachs disease and it is virtually absent in the o-variant patients. The addition of purified neuraminidase and various purified hexosaminidases exerted only a minimal synergistic effect on the hydrolysis of tay-sachs ganglioside in the lysosomal preparations from the control or patient with the o variant of tay-sachs disease. Browse pages click on an image below to see the page. View pdf of the complete article page 2339 page 2340 page 2341 page 2342 page 2343 page 2344 page 2345 citations to this article crossref (12) current issue subscribe archive rss & alerts contact copyright © 2012 american society for clinical investigation. The jci is the publication of the asci, an honor society of physician-scientists. Copying, redistribution, and other usage policies. cheap viagra online buy generic viagra cheap generic viagra cheap viagra online viagra for sale real viagra cheap viagra online viagra online buy cheap viagra viagra without a doctor prescription Se av drmdk sett 5â 677 ganger 9:59 stem cells in the treatment of macular degeneration and other ey.
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