Applied aspects Mucopolysaccharidosis is a hereditary disorder due to the accumulation of GAGs in various tissues. Examples are Hunter’s syndrome and Hurler’s syndrome.

Glycosaminoglycans belong to negatively charged heteropolysaccharides combined with a small amount of proteins. GAGs usually absorb large quantities of water and produce a gel-like matrix that forms the basis of a body’s ground substances. The viscous and lubricating properties of mucous secretion are caused by the GAGs. Hence, they are also known as mucopolysaccharides. GAGs…

our types of monosaccharides are found in all types of blood groups. They are galactose, fructose, acetylgalactosamine, and acetylglucosamine. Blood group specificity A, B, and H are associated with the non-reducing end groups of acetylglucosamine, galactose, and fructose, respectively.

This GAG does not contain any uronic acid. The polymer present here is N-acetylglucosamine with β-linkages. It is the most common heterogeneous GAG. The structure of keratan sulphate is shown in Figure 2.22. Figure 2.22 Structural Representation of Keratan Sulphate

Occurrence It is present in cornea and tendons.

Heparin is generally used to prevent blood clotting by inhibiting the conversion of prothrombin to thrombin.

Heparin consists of glucosamine N-sulphate and sulphate ester of glucuronic acid joined together by α(1 → 4) linkages (Figure 2.21). Heparin is unique in structure and is made up of mainly serine and glycine residues. Figure 2.21 Structural Representation of Heparin

Occurrence Heparin is present in combination with proteins as proteoglycan. It is one of the abundant anticoagulants present in the liver. It is also present in lungs, spleen, kidneys, and intestinal mucosa.

The structure of chondroitin is very similar to that of hyaluronic acid, as shown in Figure 2.20. The main difference in these structures N-acetylgalactosamine 6-sulphate is present in place of N-acetyl glucosamine. Figure 2.20 Structural Representation of Chondroitin Sulphate

Occurrence Chondroitin sulphate is present in cartilage, bone, heart valves, tendons, and cornea.