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Adrenoleukodystrophy

Accumulation of cholesterol esters with very long chain fatty acids

Brentwood V. A. Hospital, and the Departments of Pediatrics, Neurology and Psychiatry, UCLA Center for the Health Sciences, Los Angeles, California.

An accumulation of fatty acids having chain lengths of C₂₂ to C₂₆ was demonstrated in the cholesterol ester fraction of white matter from two boys with adrenoleukodystrophy. A similar accumulation of very long chain fatty acids was also shown in gangliosides and cerebrosides. A disorder of the one-carbon degradation system responsible for the metabolism of some of the very long chain fatty acids may be the basic enzymatic defect in this condition.

Dr. Menkes' address is 9615 Brighton Way, Beverly Hills, CA 90210.

This work was supported by grants from the National Institutes of Health (NS-12256).

Accepted for publication October 26, 1976.

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