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ASN Neuro. 2015 Feb 18;7(1). pii: 1759091415568913. doi: 10.1177/1759091415568913. Print 2015 Jan-Feb.

Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis.

Author information

1
Department of Biology, Boston College, Chestnut Hill, MA, USA.
2
Department of Biology, Boston College, Chestnut Hill, MA, USA Department of Biochemistry and Molecular Biology, Committee on Immunology, University of Chicago, IL, USA.
3
Department of Genetics, St. Jude Children's Research Hospital, Memphis, TN, USA.
4
Department of Biology, Boston College, Chestnut Hill, MA, USA seyfridt@bc.edu.

Abstract

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 in sciatic nerve. Our results indicate that the greater neurochemical pathology observed in the optic nerve than in the sciatic nerve of β-gal -/- mice is likely due to the greater glycolipid storage in optic nerve.

KEYWORDS:

X-ray diffraction; cerebrosides; gangliosides; lipids

PMID:
25694553
PMCID:
PMC4342369
DOI:
10.1177/1759091415568913
[Indexed for MEDLINE]
Free PMC Article

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