Sialosylglobotetraosylceramide: a marker for amyotropic lateral sclerosis.

Abstract

Gangliosides of healthy and pathologic muscles (amyotropic lateral sclerosis and facio-scapulo-humeral muscular dystrophy) were studied. Total ganglioside content of the affected muscles was approximately 2 fold higher than the unaffected muscles. Our results showed that ALS muscle contained a ganglioside which was absent in the unaffected and FSH muscular dystrophic muscles. Based on the results of hydrolysis with Vibrio cholerae neuraminidase and subsequent reactivity of the asialo derivative towards anti-globotetraosylceramide, we propose that the ALS ganglioside is sialosylglobtetraosylceramide, NeuAc(alpha 2-3)Ga1NAc(beta 1-3)Ga1(alpha 1-4)Ga1(beta 1-4)G1c-Cer.

• Authors: S K Kundu, Y Harati, L K Misra
• Journal: Biochemical and biophysical research communications (Biochem Biophys Res Commun) Vol. 118 Issue 1 Pg. 82-9 (Jan 13 1984) ISSN: 0006-291X [Print] United States
• PMID: 6696769 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Globosides
• Glycolipids
• Glycosphingolipids
• sialosylgloboside

Topics

• Amyotrophic Lateral Sclerosis (metabolism)
• Carbohydrate Conformation
• Carbohydrate Sequence
• Chromatography, Thin Layer
• Globosides (isolation & purification)
• Glycolipids (isolation & purification)
• Glycosphingolipids (isolation & purification)
• Humans
• Muscles (metabolism, pathology)
• Reference Values