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Ganglioside Storage Diseases

New England Journal of Medicine, 1971
For almost 100 years Tay-Sachs disease was the only ganglioside storage disease known. A second inborn error of ganglioside metabolism, generalized gangliosidosis, was discovered in 1965.13,35 Three additional ganglioside storage diseases have recently been uncovered,4,3,52,66,74 bringing the total to five.36 Of these, three involve storage of ...
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Gangliosides and hearing

Biochimica et Biophysica Acta (BBA) - General Subjects, 2017
Severe auditory impairment observed in GM3 synthase-deficient mice and humans indicates that glycosphingolipids, especially sialic-acid containing gangliosides, are indispensable for hearing. Gangliosides associate with glycoproteins to form membrane microdomains, the composition of which plays a special role in maintaining the structural and ...
Jin-Ichi, Inokuchi   +3 more
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Gangliosides of Melanoma

The Journal of Dermatology, 1992
AbstractThe relationships between the ganglioside composition of melanomas and their biologic behavior were investigated. (1) The amount of GM2 and/or GD2 in melanoma cells injected into nude mice correlated with the tumor growth rate. (2) GD2 content of melanoma cell lines correlated with sensitivity to radiation and vincristine. (3) GM2 expression of
T, Tsuchida, Y, Ishibashi
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Gangliosides in Neuroblastomas

Neurochemical Pathology, 1988
Neuroblastomas from children presenting with tumors at various ages and different primary sites (abdominal, adrenals, pelvic, and thoracic) were studied. Analysis of the ganglioside patterns of 53 tumors indicated that patients who were either disease positive 2 yr following surgery or dead of disease, had significantly (p less than 0.005) less GT1b ...
C L, Schengrund, S J, Shochat
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Ganglioside headgroup dynamics

Biochimica et Biophysica Acta (BBA) - Biomembranes, 1980
Gangliosides, spin-labelled specifically on N-acetylneuraminic acid residues or on random-headgroup sugars, have been used to extend previous studies of headgroup behaviour. Headgroup sugar mobility is seen to be homogeneous and relatively unrestricted in a range of systems including three lines of cultured cells. The effects of temperature and pH have
P M, Lee   +3 more
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Human placenta gangliosides

Lipids, 1988
AbstractGangliosides extracted from human placenta were composed of four major components (G1–G4), which were purified by silica beads column chromatography. The structures of these gangliosides were analyzed by exoglycosidase treatments, 400 MHz proton magnetic resonance spectroscopy and gas chromatography‐mass spectrometry and were concluded to be as
T, Taki   +6 more
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Mouse liver gangliosides

Carbohydrate Research, 1986
The major gangliosides from mouse liver were purified and characterized by t.l.c., g.l.c., sialidase treatment, and a methylation study. GM3(NeuAc), GM3(NeuGc), GM2(NeuGc), GM1(NeuGc), and GDla(NeuGc, NeuGc) were identified. The structural identification of three of the gangliosides, GM2(NeuGc), GM1(NeuGc), and GDla(NeuGc, NeuGc), was supported by the ...
M, Suzuki   +4 more
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