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NEUROLOGY 1985;35:1512
© 1985 American Academy of Neurology

Adrenoleuko-dystrophy

Abnormality of "tightly bound" fatty acids in the erythrocyte membrane proteins

Yasunobu Antoku, MD, Tetsuo Sakai, MD, Ikuo Goto, MD, Sanji Miyoshino, MD, Hiroshi Iwashita, MD and Yoshigoro Kuroiwa, MD

From the Department rif Neurology (Drs. Antoku, Sakai, and Iwashitai. National Chikugo Hnspital. Chikugo City; the Department of Neuroloa (Drs. Goto and Kuroiwa). Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka; and the Department of Pediatrics (Dr. Miyobhino), Nat ional Nishi-Beppu Hospital, Beppu, Japan.

"Tightly bound" and "loosely bound" fatty acids in erythrocyte membranes were analyzed in three patients with adrenoleukodystrophy, three probable carriers, and eight controls. The ratios of C28:0 or C26:0 to C22:0 or C20:0 in the tightly bound fatty acids of three patients were significantly higher than those of controls, and the ratios in two of three probable carriers were higher than those in controls. The ratios of C26:0 to C22:0 or C20:0 in the loosely bound fatty acids of three patients and three probable carriers were also significantly higher than those of controls. Since tightly bound fatty acids in membrane proteins are found not only in erythrocyte membranes but also in myelin proteins, the abnormality of tightly bound fatty acids may be related to the demyelination in adrenoleukodystrophy.

Address correspondence and reprint requests to Dr. Antoku, Department of Neurology. Natianal Chikugo Hospital, 515 Kurakazu, Chikugo City, 833 Japan.

Accepted lor publication January 28, 1985.






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