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Chorea‐acanthocytosis: Abnormal composition of covalently bound fatty acids of erythrocyte membrane proteins
Author(s) -
Sakai Tetsuo,
Antoku Yasunobu,
Iwashita Hiroshi,
Goto Ikuo,
Nagamatsu Keiji,
Shii Hiroaki
Publication year - 1991
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410290615
Subject(s) - phospholipid , chemistry , biochemistry , phosphatidylethanolamine , fatty acid , phosphatidylserine , stearic acid , chorea , phosphatidylcholine , medicine , membrane , organic chemistry , disease
Abstract Phospholipid class, peak profile of each phospholipid class, loosely bound fatty acids, covalently (tightly) bound fatty acids of the erythrocyte membrances, and plasma fatty acids were investigated using high‐performance liquid chromatography in six patients with chorea‐acanthocytosis and 14 age‐ and sex‐matched normal control subjects. Additionally, six patients with Huntington's disease were included as disease control subjects in the study of covalently bound fatty acids. Study of covalently (tightly) bound fatty acids in erythrocyte membrane proteins after alkaline hydrolysis, hitherto undescribed in chorea‐acanthocytosis, revealed that palmitic acid (C16:0) was significantly increased and stearic acid (C18:0) was decreased in the patients with chorea‐acanthocytosis. Analyses for total covalently bound fatty acids disclosed that palmitic and docosahexaenoic (C22:6) acids were increased and stearic acid was decreased in chorea‐acanthocytosis. Phospholipid class(phosphatidylcholine, phosphatidylethanolamine, sphingomyelin, and phosphatidylserine) and peak profile of each phospholipid class from the erythrocyte membranes did not differ between the patients with chorea‐acanthocytosis and the control subjects. Of the loosely bound fatty acids, linoleic acid (C18:2) was significantly decreased in those with chorea‐acanthocytosis, which seemed to be nonspecific.