Heritable disorders of connective tissue / Victor A. McKusick.

  • Victor A. McKusick
Date:
1960
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: Heritable disorders of connective tissue / Victor A. McKusick. Source: Wellcome Collection.

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    36 Heritable Disorders of Connective Tissue Table 3. Acid Mucopolysaccharides of Connective Tissue^ I. Nonsulfated mucopolysaccharides 1. Hyaluronic acid 2. Chondroitin II. Sulfated mucopolysaccharides 1. Chondroitin sulfate A 2. Chondroitin sulfate В 3. Chondroitin sulfate С 4. Heparitin sulfate 5. Keratosulfate *From Meyer, K., Hoffman, P., and Linker, A. : The Acid Mucopolysaccharides of Con¬ nective Tissue; In Tunbridge, R. E., et al. (eds.) : Connective Tissue, A Symposium, Spring¬ field, 111., 1957, Charles С Thomas, Publisher. There are several presumably distinct acid mucopolysaccharides in connective tissue (Table 3). All are polymers of high molecular weight and contain hexo- samine and hexuronic acid moieties.Hyaluronic acid, which has been identified in synovial fluid, vitreous humor, and umbilical cord, is composed, among other moieties, of glucosamine and glucuronic acid. It is digested by hyaluronidase. Chondroitin, which has been isolated only from cornea, differs from hyaluronic acid by the replacement of glucosamine by galactosamine ; its properties are similar, however. Meyer distinguishes three chondroitin sulfates (A, B, and C) on the basis of solubility, optical rotation, and enzymatic properties. All contain galac¬ tosamine, not glucosamine. ChSA has been demonstrated in cartilage, bone, cornea, aorta, and liganientum nuchae. ChSB, in which the hexuronic acid moiety is iduronic acid, not glucuronic acid, has been isolated from skin, tendon, heart valves, ligamentum nuchae, and aorta. ChSC has been found in cartilage, um¬ bilical cord, tendons, and nucleus pulposus. Keratosulfate, the only sulfate muco¬ polysaccharide free of uronic acid, has been isolated from cornea, where it represents about 50 per cent of total mucopolysaccharide. Heparitin sulfate, which as its name indicates has chemical, physical, and other properties resembling those of heparin, has normally been found only in aorta. Metabolically mucopolysaccharides are rapidly turned over. The availability of and description of its use in the study of acid mucopolysaccharides by Djiewiatkowski-'- in 1949 has permitted some insight into problems of the metabo¬ lism of these materials. Studies of mucopolysaccharide formation in microorganisms and in cell cultures have likewise been helpful. Production of components of the ground substance in tissue culture has been observed. Metachromatic staining of connective tissues, as by toluidine blue, is a func¬ tion largely of mucopolysaccharides.^®-'^'' (Metachromasia is a term introduced by Ehrlich in 1877 to indicate the property of a tissue to stain one color when ex¬ posed to a dye of a different color.) In the mammalian organism hyaluronidase has been identified with certainty only in testis. The importance of the ground substance is evident when one considers that it must be traversed by all materials entering and leaving the cells.The con- cept'®'''^- that mucopolysaccharides function like a reactive gel of the ion-exchange resin group is an intriguing but as yet unproved concept. It is at least theoretically possible that changes in the concentration or state of polymerization might modify
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