Combined deficiencies of endosomal ganglioside neuraminidases 3 and 4 in mice cause lysosomal GM3 gangliosidosis

The sialylated glycosphingolipids gangliosides are essential to myelination, axon integrity, transmission of nervous impulses and memory consolidation. Ganglioside expression patterns in the brain are cell-specific, dynamic and maintained by highly coordinated processes of biosynthesis, trafficking, processing and catabolism.