SPHINGOGLYCOLIPIDS IN THE NERVOUS SYSTEM IN FABRY'S DISEASE |
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| Authors: | T Miyatake T Ariga |
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| Institution: | Department of Neurology and Biochemistry, Faculty of Medicine, University of Tokyo, Hongo, Tokyo, Japan |
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| Abstract: | Abstract— Two glycolipids, accumulated in the spinal ganglia of a patient with Fabry's disease were identified as: galactosyl (α1 → 4) galactosyl (β1 → 4) glucosyl(1 → 1) ceramide (CTH) and galactosyl (α1 → 4) galactosyl(1 → 1) ceramide (CDG). Only one glycolipid which had the same structure as the CTH in the spinal ganglia accumulated in the sympathetic ganglia of the patient. In the nervous system, CTH contained behenic acid (C22:0) as the major fatty acid. In the spinal ganglia, CDG also contained behenic acid as the major fatty acid. |
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