linoleic-acid and Mucopolysaccharidosis-I

Gangliosides are the most complex oligosaccharide-containing glycosphingolipids defined by the presence of sialic acid and although present in all tissues, predominate in the brain. Considering their importance in neural development, it is unsurprising that ganglioside metabolism is altered in neurodegenerative diseases. The severe form of mucopolysaccharidosis type I, Hurler syndrome (HS), is characterised by progressive loss of neuronal function through largely undefined mechanisms. Here, we sought to interrogate brain gangliosides in a murine model of HS and further, assessed whether dietary modulation of lipid metabolism effected correction of the metabolic abnormalities. The simple gangliosides, G

Topics: Animals; Brain; Diet; Fatty Acids, Essential; Female; Glycosphingolipids; Linoleic Acid; Locomotion; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mucopolysaccharidosis I