Requirement of G M2 ganglioside activator for phospholipase D activation [PDF]
Proceedings of the National Academy of Sciences, 1998 Sequence analysis of a heat-stable protein necessary for the activation of ADP ribosylation factor-dependent phospholipase D (PLD) reveals that this protein has a structure highly homologous to the previously known G M2 ganglioside activator whose deficiency results in the AB-variant of G M2 ...
S, Nakamura +10 more
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Synthesis of tri- and tetrasaccharide haptens related to the Asialo forms of the gangliosides GM2 and GM1 [PDF]
Canadian Journal of Chemistry, 1984 Synthesis of βDGalNAc(1→4)βDGal(1→4)βDGlc—O(CH2)8COOCH3 (21) and βDGal(1→3)βDGalNAc(1→4)βDGal(1→4)βDGlc—O(CH2)8COOCH3 (33) are presented starting from βDGal(1→4)βDGlc—O(CH2)8COOCH3 (1) and D-galactose. Improvements in the preparations of D-galactal triacetate (7) and tri-O-acetyl-2-azido-2-deoxy-α-D-galactopyranosyl bromide (11) are reported.
Subramaniam Sabesan, Raymond U. Lemieux
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Complexing of Glycolipids and Their Transfer between Membranes by the Activator Protein for Degradation of Lysosomal Ganglioside GM2 [PDF]
European Journal of Biochemistry, 1982 The lysosomal degradation of ganglioside GMZ by hexosaminidase A depends on the presence of the specific activator protein which mediates the interaction between micellar or membrane‐bound ganglioside and water‐soluble hydrolase. The mechanism and the glycolipid specificity of this activator were studied in more detail.
E, Conzelmann +3 more
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Incorporation and metabolism of ganglioside GM2 in skin fibroblasts from normal and GM2 gangliosidosis subjects [PDF]
European Journal of Biochemistry, 1985 Ganglioside GM2, 3H‐labeled in the sphingoid base, was added to the culture medium of normal and GM2 gangliosidosis fibroblasts. Ganglioside was found to adsorb rapidly to the cell surface, most of it could however be removed by trypsination. The trypsin‐resistant incorporation was about 10 nmol/mg cell protein, after 48 h.
S, Sonderfeld +5 more
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Increased Concentration of GM1-Ganglioside in Cerebrospinal Fluid in Dogs with GM1- and GM2-Gangliosidoses and its Clinical Application for Diagnosis [PDF]
Journal of Veterinary Diagnostic Investigation, 2004 GM1- and GM2-gangliosidoses are lethal lysosomal diseases that are caused by a defect of acid hydrolases, resulting in the intralysosomal accumulation of the specific physiological substrates, GM1- and GM2-gangliosides, respectively. In the present study a method for the diagnosis of canine GM1-gangliosidosis was established using canine cerebrospinal
Hiroyuki, Satoh +4 more
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Mice doubly-deficient in lysosomal hexosaminidase A and neuraminidase 4 show epileptic crises and rapid neuronal loss. [PDF]
PLoS Genetics, 2010 Tay-Sachs disease is a severe lysosomal disorder caused by mutations in the HexA gene coding for the α-subunit of lysosomal β-hexosaminidase A, which converts G(M2) to G(M3) ganglioside. Hexa(-/-) mice, depleted of β-hexosaminidase A, remain asymptomatic
Volkan Seyrantepe +15 more
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Clostridium perfringens delta toxin is sequence related to beta toxin, NetB, and Staphylococcus pore-forming toxins, but shows functional differences. [PDF]
PLoS ONE, 2008 Clostridium perfringens produces numerous toxins, which are responsible for severe diseases in man and animals. Delta toxin is one of the three hemolysins released by a number of C. perfringens type C and possibly type B strains.
Maria Manich +7 more
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Molecules, 2012 Glycosphingolipids from the ganglio-series are usually classified in four series according to the presence of 0 to 3 sialic acid residues linked to lactosylceramide.
Agata Steenackers +8 more
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Journal of Lipid Research, 1972 Analyses have been made of glycosphingolipids from visceral organs and brain of a patient with an unusual lipid storage disorder diagnosed initially as classical Tay-Sachs disease. Levels of the lipids from fresh-frozen sections of gray and white matter, kidney, spleen, liver, and heart from this patient were compared with those of normal juvenile ...
Paul D. Snyder, Jr. +2 more
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