Myelin Abnormalities in the Optic and Sciatic Nerves in Mice With GM1-Gangliosidosis

GM1-gangliosidosis is a glycosphingolipid lysosomal storage disease involving accumulation of GM1 and its asialo form (GA1) primarily in the brain. Thin-layer chromatography and X-ray diffraction were used to analyze the lipid content/composition and the myelin structure of the optic and sciatic nerves from 7- and 10-month old β-galactosidase (β-gal) +/? and β-gal –/– mice, a model of GM1gangliosidosis. Optic nerve weight was lower in the β-gal –/– mice than in unaffected β-gal +/? mice, but no difference was seen in sciatic nerve weight. The levels of GM1 and GA1 were significantly increased in both the optic nerve and sciatic nerve of the β-gal –/– mice. The content of myelin-enriched cerebrosides, sulfatides, and plasmalogen ethanolamines was significantly lower in optic nerve of β-gal –/– mice than in β-gal +/? mice; however, cholesteryl esters were enriched in the β-gal –/– mice. No major abnormalities in these lipids were detected in the sciatic nerve of the β-gal –/– mice. The abnormalities in GM1 and myelin lipids in optic nerve of β-gal –/– mice correlated with a reduction in the relative amount of myelin and periodicity in fresh nerve. By contrast, the relative amount of myelin and periodicity in the sciatic nerves from control and β-gal –/– mice were indistinguishable, suggesting minimal pathological involvement...
Source: ASN Neuro - Category: Neuroscience Authors: Tags: Original Article Source Type: research