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SPHINGOGLYCOLIPIDS IN THE NERVOUS SYSTEM IN FABRY'S DISEASE
Author(s) -
Miyatake T.,
Ariga T.
Publication year - 1972
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1972.tb01479.x
Subject(s) - behenic acid , glycolipid , ceramide , nervous system , fatty acid , chemistry , biochemistry , biology , neuroscience , apoptosis , palmitic acid
— 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 (C 22:0 ) as the major fatty acid. In the spinal ganglia, CDG also contained behenic acid as the major fatty acid.