Ganglioside synthases

Fig. 5B). The elevation of ganglioside synthesis was confirmed to be due, in part at least, to increased activity of three gangliosides synthases, as assayed with lysates prepared from the cells pretreated with L-PDMP as described above. 

The promoter of STSSia I (GD3-synthase, STII), which has attracted much more attention than have other promoters of the ganglioside synthase genes, has been cloned from rat [103], mouse [104] and human [105]. The promoter of all three species is TATA-less and does not possess known core- promoter elements, including the initiator (INR), the downstream promoter (DPE), the TFIIB recognition elements (BRE), and the motif ten element (MTE). Similar to other TATA-less promoters, it has a high GC content and is enriched in Spl-bind-ing sites. The proximal promoter region is defined within 500 or 700 bp upstream of the ATG... 

Yamamoto A, Haraguchi M, Yamashiro S, Fuku-moto S, Funikawa K, Takamiya K, Atsuta M, Shiku H, Funikawa K (1996) Heterogeneity in the expression pattern of two ganglioside synthase genes during mouse brain development. J Neurochem 66 26-34... 

RNA interference-mediated silencing of GM3 synthase protects HT22 cells from glutamate-mediated cell death. Collective evidence suggests that GM1 and GM3 gangliosides act very differently in brain tissue and more studies are required on the molecular mechanism of action of various gangliosides in brain tissue. 

Mizutani, A., Kuroda, Y., Muramoto, K., Kobayashi, K., Yamagishi, K., and Inokuchi, J. (1996). Effects of glucosylceramide synthase inhibitor and ganglioside GCMb on synchronous oscillations of intracellular Ca2+ in cultured cortical neurons. Biochem. Biophys. Res. Commun. 222, 494—498. 

M. Wendeler, H. Reiiaender, J. Hoernschemeyer, G. Schwarzmann, T. Kolter, K. Sandhoff, Recombinant Ganglioside GM2-Synthase - Expression in Insect Cells and Enzyme Assay, Y. C. Lee, R. T. Lee, eds., Methods in Enzymology Recognition of Carbohydrates in Biological Systems, submitted for publication. 

Ishii A, Ohta M, Watanabe Y, Matsuda K, Ishiyama K, Sakoe K, Nakamura M, Inokuchi J, Sana Y, Saito M. Expression cloning and functional characterization of human cDNA for ganglioside GM3 synthase. J. Biol. Chem. 1998 273 31652-31655. 

Unlike lysosomal lipid-storage diseases (see below), very few metabolic diseases are known to be related to mutations of glycosyltransferases in ganglioside synthesis. The one new finding has been from that of Simpson et al. [45], who showed that a nonsense mutation of ST-I (GM3-synthase) is the cause of human autosomal recessive infantile-onset s)mptomatic epilepsy s)mdrome. Because ST-I is a key enz)me in the synthesis of all complex gangliosides, this report clearly indicates that the precise expression of complex gangliosides has critical biological functions in human nervous system development. 

Chung TW, Choi HJ, Lee YC, Kim CH (2005) Molecular mechanism for transcriptional activation of ganglioside GM3 synthase and its function in differentiation of HL-60 cells. Glycobiology 15 233-244... 

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