Occurrence of Lysoganglioside Lyso-GM2(II3-Neu5Ac-Gangliotriaosylsphingosine) in GM2Gangliosidosis Brain
- 1 January 1986
- journal article
- research article
- Published by Walter de Gruyter GmbH in Biological Chemistry Hoppe-Seyler
- Vol. 367 (1) , 241-244
- https://doi.org/10.1515/bchm3.1986.367.1.241
Abstract
Lysoganglioside lyso-GM2 (sialylgangliotriaosylsphingosine) was detected in a brain sample of a patient with variant B of infantile GM2 gangliosidosis (Tay-Sachs disease) at a concentration of approx. 15 nmol/g wet tissue. Neither lysoganglioside lyso-GM2 nor the corresponding GM1 derivative could be detected in normal brain.This publication has 12 references indexed in Scilit:
- Krabbe disease: a galactosylsphingosine (psychosine) lipidosis.Published by Elsevier ,2021
- Progressive Accumulation of Toxic Metabolite in a Genetic LeukodystrophyScience, 1984
- The Protein Activator Specific for the Enzymic Hydrolysis of GM2Ganglioside in Normal Human Brain and Brains of Three Types of GM2GangliosidosisJournal of Neurochemistry, 1983
- The occurrence of psychosine and other glycolipids in spleen and liver from the three major types of gaucher's diseaseBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1982
- Deficiency of the Hexosaminidase A Activator Protein in a Case of GM2 Gangliosidosis; Variant ABPediatric Research, 1982
- AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2.Proceedings of the National Academy of Sciences, 1978
- Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidaseBiochemical and Biophysical Research Communications, 1972
- ENZYME ALTERATIONS AND LIPID STORAGE IN THREE VARIANTS OF TAY‐SACHS DISEASEJournal of Neurochemistry, 1971
- Use of Butyl Acetate in Determination of Sialic Acid.Acta Chemica Scandinavica, 1959
- Quantitive estimation of sialic acidsBiochimica et Biophysica Acta, 1957