Chorea-acanthocytosis: abnormal composition of covalently bound fatty acids of erythrocyte membrane proteins.
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.
Sakai
T
T
Department of Neurology, National Chikugo Hospital, Fukuoka, Japan.
Antoku
Y
Y
Iwashita
H
H
Goto
I
I
Nagamatsu
K
K
Shii
H
H
D000050:Acanthocytes / Q000737:chemistry*; D000328:Adult; D002819:Chorea / Q000097:blood* / Q000235:genetics; D004910:Erythrocyte Membrane / Q000737:chemistry*; D005227:Fatty Acids / Q000097:blood*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000097:blood; D019787:Linoleic Acid; D008041:Linoleic Acids / Q000097:blood; D008297:Male; D008563:Membrane Lipids / Q000032:analysis*; D008565:Membrane Proteins / Q000032:analysis*; D008875:Middle Aged; D019308:Palmitic Acid; D010169:Palmitic Acids / Q000097:blood; D010743:Phospholipids / Q000097:blood*; D013229:Stearic Acids / Q000097:blood; D013577:Syndrome
D005227:Fatty Acids; D008041:Linoleic Acids; D008563:Membrane Lipids; D008565:Membrane Proteins; D010169:Palmitic Acids; D010743:Phospholipids; D013229:Stearic Acids; D019308:Palmitic Acid; C031183:stearic acid; D019787:Linoleic Acid