Use of isotopically radiolabelled GM3 ganglioside to study metabolic alterations in Salla disease.

Abstract

We report the preparation of radioactive GM3 ganglioside and its use in the study of sialic acid storage disorders. For the first time GM3 was isotopically radiolabeled in three positions of the molecule: at the sialic acid acetyl group, [3H-Neu5Ac]GM3, at the C1 of the fatty acid moiety, [14C-Stearoyl]GM3, and at C3 of sphingosine, [3H-Sph]GM3. The radioactive GM3 administered to cultured human fibroblasts from a patient suffering from Salla disease was taken up by the cells and metabolized. An analysis of the distribution of radioactivity within the ganglioside metabolic derivatives showed an accumulation of free sialic acid and ceramide in the pathological cells.

• Authors: V Chigorno, M Valsecchi, M Nicolini, S Sonnino
• Journal: Indian journal of biochemistry & biophysics (Indian J Biochem Biophys) 1997 Feb-Apr Vol. 34 Issue 1-2 Pg. 150-6 ISSN: 0301-1208 [Print] India
• PMID: 9343943 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Carbon Radioisotopes
• G(M3) Ganglioside
• Sialic Acids
• Tritium

Topics

• Animals
• Carbohydrate Sequence
• Carbon Radioisotopes
• Cattle
• Cells, Cultured
• G(M3) Ganglioside (chemistry, metabolism)
• Humans
• Lysosomal Storage Diseases (metabolism)
• Molecular Sequence Data
• Molecular Structure
• Sialic Acids (metabolism)
• Tritium