Phospholipid class, peak profile of each
phospholipid class, loosely bound
fatty acids, covalently (tightly) bound
fatty acids of the erythrocyte membranes, 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.