Glycosaminoglycans laboratory

A program in our laboratory on the chemistry of mucopolysaccharides (glycosaminoglycans) has been greatly enhanced by the availability of 13c-NMR. For instance, its application (10,19,... 

Pennock CA (1976) A review and selection of simple laboratory methods used for the study of glycosaminoglycan excretion and the diagnosis of the mucopolysaccharidoses. J Clin Pathol 29 111-123... 

Based on our knowledge of the facts that (a) lipoproteins are carriers of cholesterol in the blood stream, (b) they are involved in atherogenesis,(c) pectin when supplemented in diet causes lowering of serum and/or liver cholesterol in man as well as a number of laboratory animals and (d) polyanionic glycosaminoglycans interact with lipoproteins, it was of interest to us to investigate the interaction of polyanionic pectin with lipoproteins in order to explain the biochemical basis by which pectin may cause lowering of serum/liver cholesterol levels. 

Human skin equivalents have been developed by several laboratories. One equivalent, Testskin, consists of human keratinocytes seeded onto a collagen base or collagen-glycosaminoglycan matrix containing human fibroblasts. In many respects, the epidermis which develops resembles epidermis in vivo. The tissue culture system survives for several weeks and may be useful in studying skin penetration. Testskin is a commercially produced skin equivalent system marketed by Organogenesis, Inc. (Cambridge, MA) ... 

A 15-year-old boy has a long history of school problems and is labeled as hyperactive. His tissues are puffy, giving his face a coarse appearance. His IQ tests have declined recently and are now markedly below normal. Laboratory studies demonstrate normal amounts of sphin-golipids in fibroblast cultures with increased amounts of glycosaminoglycans in urine. Which of the following enzyme deficiencies might explain the boy s phenotype ... 

Several inherited diseases of connective tissues are known (McKusick, 1966), some of which involve a derangement of glycosaminoglycan metabolism, and, in particular, Hurler s syndrome has been extensively studied in many laboratories (Dorfman, 1966a Dorfman and Matalon, 1969). The disorder expresses itself biochemically as an accumulation of glycosaminoglycans in various tissues (particularly dermatan sulfate and heparan sulfate), and recent work by Fratantoni si al. (1968) and Neufeld and Fratantoni (1970) suggests that there is a defect in degradation of the glycosaminoglycans rather than an overproduction. 

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