Fish oil and myelin
- 1 July 1998
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 51 (1) , 5-7
- https://doi.org/10.1212/wnl.51.1.5
Abstract
Peroxisomal disorders can be divided into two major categories. The first include X-linked adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN), in which the peroxisome is structurally intact, but a single biochemical defect occurs. In contrast, disorders of peroxisome biogenesis are a group of diseases resulting from defective organelle assembly, and hence, multiple peroxisome enzyme abnormalities, chiefly defects in beta oxidation of very-long-chain fatty acids (VLCFA) and in synthesis of essential lipids such as plasmalogens.2 ZS is a member of this second category, along with neonatal adrenoleukodystrophy (NALD) and infantile Refsum's disease (IRD), both of which were named before their relation to peroxisomal disturbances was recognized. Recently, it has been shown that these three syndromes typically are associated with defects in genes encoding for an integral peroxisome membrane protein, a receptor involved in targeting proteins toward the peroxisome, or an ATPase required for stability of this receptor.3-5 Biochemical and complementation studies have provided further evidence that ZS, NALD, and IRD represent a single disease entity with variations in degree of biochemical disturbances and age of onset and, thus, clinical …Keywords
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