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249, 7969-7976. Norman, R. , Tingey, A. H. & Goodbody, R. A. (1959) Tay-Sachs disease with visceral involvement and its relationship to Niemann-Pick's disease. / . Path. Bact. 78, 409^21. O'Brien, J. S. (1975) Molecular genetics of G M 1 jft-galactosidase. Clin. Genet. 8, 303-313. O'Brien, J. , Stern, M. , Landing, B. , O'Brien, J. K. & Donnell, G. N. (1965) Generalized gangliosidosis. Amer. J. Dis. Child. 109, 338-346. , Ho, M. , Veath, M. , Wilson, J. , Opitz, J. , ZuRhein, G. , Spranger, J. , Hartman, H.

Some cells may be clear, whereas others may be faintly yellow and still others, a dark brown or olive green. It is possible that a number of reported ceroid storage diseases are Niemann-Pick's disease. Although it has been speculated that the ceroid and lipofuscin pigments may be formed as a result of oxidation of unsaturated lipids by hydrogen peroxide or superoxide anions, the formation of ceroid and lipofuscin-like bodies in Niemann-Pick disease and its relationship to impaired sphingomyelin catabolism is not known.

1935) Uber die Natur der Phosphatide und anderer lipoide des Gehirns und der Leber bei der Niemann-Pickschen Krankheit. Hoppe-Seylers Z. Physiol. Chem. 235, 24-36. Klenk, E. & Schumann, E. (1942) Uber die Ganglioside, des Gehirns bei der infantilen amaurotischen Idiotie von Typus Tay-Sachs. Ber Deutsch. Chem. Ges. 75, 1632-1636. Kolodny, E. , Brady, R. O & Volk, B. W. (1969) Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease. Biochem. Biophys. Res. Commun. 37, 526-531.

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