Accumulation of hydrocarbon in a patient with adrenoleukodystrophy

Abstract

Summary The lipid composition of tissues from a patient with adrenoleukodystrophy was examined. Cholesterol esters of both brain and adrenal tissues contained increased proportions of fatty acids longer than C₂₂ in agreement with previous reports. During this study, thin-layer chromatographic analysis of the neutral lipid fraction from the cerebral white matter and the adrenal gland revealed an unknown spot, which was identified as hydrocarbon of n-alkanes (C_nH_2n+2, n=16–33) by means of gas-liquid chromatography and gas chromatography/mass spectrometry. There may be a relationship between the accumulation of hydrocarbons and the change in fatty acid metabolism that is the fundamental disorder of ALD, as: (1) the fatty acids abnormally accumulated in cerebral white matter and adrenal gland coincided in chain length (i.e. C₂₄–C₂₆) with the main peaks of n-alkane in the same tissues, and (2) an abnormal accumulation of n-alkanes occurred in the diseased tissues where cholesterol esters with very long chain fatty acids were markedly increased.