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VII. Elevated Sulfated Acid Polysaccharide Levels in Urine and Postmortem Tissues

MARGARET BISCHEL, MD; JAMES AUSTIN, MD; MATTHIAS KEMENY

Arch Neurol. 1966;15(1):13-28.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

ACID polysaccharides (APS)* have become a focus of general biomedical interest since large excesses were reported in Hurler's syndrome (gargoylism).^1,2,7 Large excesses of APS have also been noted in urine of two children who had a form of metachromatic leukodystrophy (MLD).³ Apart from their relationships with diseases, the APS in the normal nervous system have negative charge characteristics which may be of neurophysiological interest. For example, the net charge of APS molecules might influence the distribution of cations and positively charged molecules. In this manner APS might have some general bearing on the bioelectric potentials involved in neuronal excitation and transmission.

The purpose of the present study is three-fold: (1) to document and extend the APS observations in MLD; (2) to correlate them with the excess APS isolated from the brain and kidney of the same MLD children; and (3) to review a sizable expansion of the . . . [Full Text PDF of this Article]

Author Affiliations
PORTLAND, ORE

From the Division of Neurology, University of Oregon Medical School, Portland.

Footnotes
Submitted for publication Jan 24, 1966; accepted Feb 23.

In the present study ethanol fractionation techniques are used to obtain from one to three different APS fractions (Table 8). These fractions are designated APS I, APS II, and APS III. If keratosulfate or chondroitin sulfate B are present within these fractions, they are each identifiable chromatographically.¹⁵ With the chromatographic methods now available, the chondroitin sulfate A and C isomers are indistinguishable. Hence, a spot which could be either is categorized herein as "chondroitin sulfate A/C." Heparin and heparitin sulfate are not distinguishable chromatographically without prior hydrolysis. A spot which could be either is thus categorized as "heparin/heparitin sulfate." Less well-categorized APS moieties have also been encountered in the present study. On the basis of their chromatographic migration rates, these fall into two provisional groups: "unclassified group I" (of lower Rf) and "unclassified group II" (of higher Rf).15

Glycosaminoglycans is another name proposed for the acid polysaccharides (APS) considered here. This term emphasizes that these polysaccharides contain aminosugars. Other terminology for the APS studied here would be: keratosulfate=keratan sulfate; chondroitin sulfate A=chondroitin-4-sulfate; chondroitin sulfate B chondroitin sulfate C=chondroitin-6-sulfate; heparitin sulfate= heparan monosulfate.

Reprint requests to Division of Neurology, University of Oregon Medical School, Portland, Ore 97201 (Dr. Bischel).

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