Use of isotopically radiolabelled GM3 ganglioside to study metabolic alterations in Salla disease.
Indian J Biochem Biophys
;
1997 Feb-Apr; 34(1-2): 150-6
Article
in English
| IMSEAR
| ID: sea-26781
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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Sialic Acids
/
Tritium
/
Humans
/
Cattle
/
Carbon Radioisotopes
/
Molecular Sequence Data
/
Molecular Structure
/
Cells, Cultured
/
Carbohydrate Sequence
/
Lysosomal Storage Diseases
Language:
English
Journal:
Indian J Biochem Biophys
Year:
1997
Type:
Article
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