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Review
. 1992;15(4):634-44.
doi: 10.1007/BF01799620.

X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect

R J Wanders  1 , C W van Roermund, W Lageweg, B S Jakobs, R B Schutgens, A A Nijenhuis, J M Tager
Affiliations
Review

X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect

R J Wanders et al. J Inherit Metab Dis. 1992.

Abstract

The adrenoleukodystrophies refer to three genetically distinct disorders all characterized by the accumulation of very long-chain fatty acids. In this paper we will review the biochemical aspects of these leukodystrophies with particular emphasis on the methods used to measure very long-chain fatty acid levels in plasma and their reliability. Furthermore, we will concentrate on the primary defect in the X-linked form of adrenoleukodystrophy.

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