Meta-Biol

• Pathways

Gangliosides are part of the larger family of glycosphingolipids and are components of the synaptic plasma membrane involved in synaptic plasticity, signal transduction and endocytosis, processes for CNS development. Complex gangliosides (G) can be mono- (M), di- (D), and tri- (T) sialic acid-containing glycosphingolipids generated by sequential glycosylations in the ER and Golgi. Beta-1,4 N-acetylgalactosaminyltransferase 1 (B4GALNT1), a homodimeric protein (Li et al. 2000) residing on the Golgi membrane, catalyses the transfer of N-acetyl-galactosamine (GalNAc) into GM3, GD3 (and globotriaosylceramide, not shown here) by a beta-1,4 linkage (Nagata et al. 1992). Defects in B4GALNT1 can cause spastic paraplegia 26 (SPG26), a neurodegenerative disorder characterised by a slow, gradual, progressive weakness and spasticity of the lower limbs (Boukhris et al. 2013).