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Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis).
J Lipid Res 1967; 8(2):105-13JL

Abstract

The carbohydrate composition was determined for ceramide hexosides isolated from brains of patients with Tay-Sachs disease and generalized gangliosidosis (hereby named GM1-gangliosidosis). Gray matter of patients with each disease showed a characteristic abnormal ceramide hexoside pattern. In Tay-Sachs gray matter, ceramide trihexoside is the major component, whereas ceramide tetrahexoside is barely detectable. In GM1-gangliosidosis, ceramide tetrahexoside is the major ceramide hexoside, while ceramide trihexoside is present only in small amount. These two major components have been characterized as the asialo derivatives of, respectively, the "Tay-Sachs ganglioside" (GM2-ganglioside) and the normal major monosialoganglioside (GM1-ganglioside). In both diseases, more than half the ceramide monohexoside of gray matter was glucocerebroside. Gray matter ceramide dihexoside, present in both diseases at higher than normal levels, was mostly ceramide lactoside, with possibly a small amount of ceramide digalactoside. Sulfatide contained only galactose. The abnormal ceramide hexoside pattern is limited to gray matter: white matter showed normal ceramide hexosides, i.e. a preponderance of monohexosides and sulfatide, with no detectable glucocerebroside.

Authors+Show Affiliations

The Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, New York 10461, USA.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

14564716

Citation

Suzuki, K, and G C. Chen. "Brain Ceramide Hexosides in Tay-Sachs Disease and Generalized Gangliosidosis (GM1-gangliosidosis)." Journal of Lipid Research, vol. 8, no. 2, 1967, pp. 105-13.
Suzuki K, Chen GC. Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis). J Lipid Res. 1967;8(2):105-13.
Suzuki, K., & Chen, G. C. (1967). Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis). Journal of Lipid Research, 8(2), pp. 105-13.
Suzuki K, Chen GC. Brain Ceramide Hexosides in Tay-Sachs Disease and Generalized Gangliosidosis (GM1-gangliosidosis). J Lipid Res. 1967;8(2):105-13. PubMed PMID: 14564716.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (GM1-gangliosidosis). AU - Suzuki,K, AU - Chen,G C, PY - 1967/3/1/pubmed PY - 2003/12/10/medline PY - 1967/3/1/entrez SP - 105 EP - 13 JF - Journal of lipid research JO - J. Lipid Res. VL - 8 IS - 2 N2 - The carbohydrate composition was determined for ceramide hexosides isolated from brains of patients with Tay-Sachs disease and generalized gangliosidosis (hereby named GM1-gangliosidosis). Gray matter of patients with each disease showed a characteristic abnormal ceramide hexoside pattern. In Tay-Sachs gray matter, ceramide trihexoside is the major component, whereas ceramide tetrahexoside is barely detectable. In GM1-gangliosidosis, ceramide tetrahexoside is the major ceramide hexoside, while ceramide trihexoside is present only in small amount. These two major components have been characterized as the asialo derivatives of, respectively, the "Tay-Sachs ganglioside" (GM2-ganglioside) and the normal major monosialoganglioside (GM1-ganglioside). In both diseases, more than half the ceramide monohexoside of gray matter was glucocerebroside. Gray matter ceramide dihexoside, present in both diseases at higher than normal levels, was mostly ceramide lactoside, with possibly a small amount of ceramide digalactoside. Sulfatide contained only galactose. The abnormal ceramide hexoside pattern is limited to gray matter: white matter showed normal ceramide hexosides, i.e. a preponderance of monohexosides and sulfatide, with no detectable glucocerebroside. SN - 0022-2275 UR - https://www.unboundmedicine.com/medline/citation/14564716/Brain_ceramide_hexosides_in_Tay_Sachs_disease_and_generalized_gangliosidosis__GM1_gangliosidosis__ L2 - http://www.jlr.org/cgi/pmidlookup?view=long&pmid=14564716 DB - PRIME DP - Unbound Medicine ER -