Sulfatides and Demyelination
- 1 July 1969
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 21 (1) , 32-43
- https://doi.org/10.1001/archneur.1969.00480130046005
Abstract
METACHROMATIC leukodystrophy (MLD) is a progressive, demyelinating disorder which is characterized chemically by the accumulation of a class of acidic glycolipids: the sulfatides. Pathologically, demyelination involves both the central nervous system (CNS) and peripheral nervous system, and granular, metachromatic deposits are spread throughout the neuraxis. Similar metachromatic granular material is found both intracellularly and as deposits in the tubules of the kidneys, adrenal glands, gallbladder, and liver.1 Austin et al2first suggested the presence of an enzyme defect in MLD, and Mehl and Jatzkewitz,3after demonstrating desulfation of sulfatides to cerebrosides in vitro, have shown a deficiency in a degradative enzyme system, cerebroside sulfatase, in kidney tissue from a patient with MLD.4,5Moser et al6studied the turnover of injected sodium sulfate S-35 in patients with MLD. In comparison with control groups, a greatly prolonged isotope turnover was found in MLD. They concluded thatKeywords
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