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Science 10 January 1975:
Vol. 187. no. 4171, pp. 68 - 70
DOI: 10.1126/science.803227
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Articles

Science, Vol 187, Issue 4171, 68-70
Copyright © 1975 by American Association for the Advancement of Science

articles

Deficient Ganglioside Biosynthesis: a novel human sphingolipidosis

PH Fishman, Max SR, JF Tallman, RO Brady, NK Maclaren, and M Cornblath

An unusual lipid storage disese is chracterized by the accumulation of hematoside (Gms3) in the patient's liver and brain. In contrast to the other sphingoliidoses, the accumulation of Gm3 is not the result of a defective catabolic reaction, but is the first disorder caused by deficiency in ganglioside biosynthesis to be described in man.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Ganglioside depletion and EGF responses of human GM3 synthase-deficient fibroblasts.
Y. Liu, Y. Su, M. Wiznitzer, O. Epifano, and S. Ladisch (2008)
Glycobiology 18, 593-601
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Evidence of Genetic Heterogeneity for Hereditary Gingival Fibromatosis.
T.C. Hart, D. Pallos, L. Bozzo, O.P. Almeida, M.L. Marazita, J.R. O'Connell, and J.R. Cortelli (2000)
Journal of Dental Research 79, 1758-1764
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Biosynthesis and function of gangliosides.
P. Fishman and R. Brady (1976)
Science 194, 906-915
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Inherited metabolic diseases of the nervous system.
R. Brady (1976)
Science 193, 733-739
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Biochemical Genetics in Neurology.
R. O. Brady (1976)
Arch Neurol 33, 145-151
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