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Genetic control of ganglioside biosynthesis in mice

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Abstract

The enzymatic basis for the differences in hepatic ganglioside patterns in the mouse strains C57Bl/6 and Swiss White (SW) was investigated. SW has a “Swiss-type” ganglioside profile, expressing GM1 and GD1a in addition to GM2 as major hepatic gangliosides, whereas C57Bl/6 shows a “GM2-type” profile, expressing only GM2 as the major hepatic ganglioside. The enzyme UDP-galactose:GM2 ganglioside galactosyltransferase (GM2-GalT), which catalyzes the synthesis of GM1 ganglioside, showed a four- to fivefold elevation in intact and solubilized liver Golgi membrane fractions of the SW strain compared to C57Bl/6. Crosses between C57Bl/6 and SW produced an F1 generation with a hepatic ganglioside and enzymatic phenotype intermediate between those of the two parental strains. All three genotypic groups show two forms of the Golgi apparatus enzyme with isoelectric points of 6.5–6.8 and 8.3–9.0. The simplest mode of action of genes which control the enzymatic phenotype that would be consistent with these findings are one or two structural genes or one or two cis-regulatory genes affecting the rate of enzyme synthesis.

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This work was supported by a grant from the Medical Research Council (Canada) to its Human Genetics Research Group. D.S. thanks the MRC and the McGill University faculty of medicine for studentship support.

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Sokoloff, D., Hechtman, P. Genetic control of ganglioside biosynthesis in mice. Biochem Genet 26, 631–644 (1988). https://doi.org/10.1007/PL00020502

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  • DOI: https://doi.org/10.1007/PL00020502

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