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

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dc.contributor.authorChigorno, Ven_US
dc.contributor.authorValsecchi, Men_US
dc.contributor.authorNicolini, Men_US
dc.contributor.authorSonnino, Sen_US
dc.date.accessioned1997-02-01en_US
dc.date.accessioned2009-05-27T09:12:11Z
dc.date.available1997-02-01en_US
dc.date.available2009-05-27T09:12:11Z
dc.date.issued1997-02-01en_US
dc.description.abstractWe 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.en_US
dc.description.affiliationDepartment of Medical Chemistry and Biochemistry, Medical School, University of Milan, Italy.en_US
dc.identifier.citationChigorno V, Valsecchi M, Nicolini M, Sonnino S. Use of isotopically radiolabelled GM3 ganglioside to study metabolic alterations in Salla disease. Indian Journal of Biochemistry & Biophysics. 1997 Feb-Apr; 34(1-2): 150-6en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/26781
dc.language.isoengen_US
dc.source.urihttps://https://www.niscair.res.in/ScienceCommunication/ResearchJournals/rejour/ijbb/ijbb0.aspen_US
dc.subject.meshAnimalsen_US
dc.subject.meshCarbohydrate Sequenceen_US
dc.subject.meshCarbon Radioisotopesen_US
dc.subject.meshCattleen_US
dc.subject.meshCells, Cultureden_US
dc.subject.meshG(M3) Ganglioside --chemistryen_US
dc.subject.meshHumansen_US
dc.subject.meshLysosomal Storage Diseases --metabolismen_US
dc.subject.meshMolecular Sequence Dataen_US
dc.subject.meshMolecular Structureen_US
dc.subject.meshSialic Acids --metabolismen_US
dc.subject.meshTritiumen_US
dc.titleUse of isotopically radiolabelled GM3 ganglioside to study metabolic alterations in Salla disease.en_US
dc.typeJournal Articleen_US
dc.typeResearch Support, Non-U.S. Gov'ten_US
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