Mucopolysaccharides, storage

Hall CW, Liebaers I, Di Natale P, Neufeld EF (1978) Enzymic diagnosis of the genetic mucopolysaccharide storage disorders. Methods Enzymol 50 439-456... 

Kresse H, von Figura K, Klein U, Glossl J, Paschke E, Pohlmann R (1982) Enzymic diagnosis of genetic mucopolysaccharide storage disorders. Methods Enzymol 83 559-572... 

Carbohydrates in algae and plants are often classified based on methodological discrimination. The structural carbohydrates are not water-soluble, whereas the other types of carbohydrates are water-soluble and typically extracted by hot water. In Phaeocystis five different pools of carbohydrates can be distinguished. Like all algal and plant cells, both solitary and colonial cells produce (1) structural carbohydrates, polysaccharides that are mainly part of the cell wall, (2) mono- and oligosaccharides, which are present as intermediates in the synthesis and catabolism of cell components, and (3) intracellular storage glucan. Colonial cells of Phaeocystis excrete (4) mucopolysaccharides, heteropolysaccharides that... 

In addition to a structural function in the mucous matrix, mucopolysaccharides were also suggested to serve as storage polysaccharides as nocturnal consumption of extracellular polysaccharides was reported in P. globosa (Lancelot and Mathot 1985 Veldhuis and Admiraal 1985). In both studies cells were separated from mucous matrix by filtration under pressure. Since this... 

B15. Brown, D. H., Tissue storage of mucopolysaccharide in Hiirler-Pfaundler s disease. Proc. Natl. Acad. Set. U. S. 43, 783-790 (1957). 

Gll. Grumbach, M. M., and Meyer, K., Urinary excretion and tissue storage of sulfated mucopolysaccharides in Hurler s syndrome. A.M.A. ]. Diseases Children 96, 467-469 (1958). 

Mucolipidoses are characterized by a combined metabolic disorder of mucopolysaccharides, lipids, and glycoproteins. Lysosomal storage and foamy swollen Kupffer cells with hepatomegaly may be seen. In some of the numerous types, the underlying enzymatic defects have not yet been detected. Type II is also called Leroy syndrome (J.G. Leroy et al., 1967). Due to distinctive cytoplasmic inclusions in fibroblast cultures, this disorder is also known as inclusion cell disease (J.G. Leroy et al., 1971). Foamy altered stellate cells, macrophages and also epithelioid foam cell granulomas are found. 

M46. Muir, H., Structure and enzymic degradation of mucopolysaccharides. In Lysosomes and Storage Diseases (H. G. Hers and F. Van Hoof, eds.), pp. 79-104. Academic Press, New York, 1973. 

Hemosiderin an iron storage protein of the mammalian organism, functionally related to Ferritin (see). H. is deposited in the liver and spleen (hemosiderosis), particularly in diseases associated with increased blood destruction, such as pernicious anemia, or with increased iron resorption (hemochromatosis), or even in hemorrhages Most of the deposits are located in the liver, which may contain up to 50 g H., compared with the normal content of 120 to 300 mg H. H. from horse spleen consists of 26-34 % iron(III), and up to 35 % protein (aposiderin). The rest is made up of octasubstituted porphyrin, mucopolysaccharides and fatty acid esteis. 

Keratan sulfate an acidic mucopolysaccharide composed of N-acetyl-o-glucosamine 6-sulfate and D-galactose, linked alternately by P-1,3 and P-1,4 glyco-sidic bonds. K.s. is found in the cornea of the eye, in cartilage, in the aorta and in intervertebral discs. For formula, see Lysosomal storage diseases (Mucopolysaccharidoses). 

Since ganglioside storage is observed in gargoylism (Pfaundler-Hurler syndrome), this disease will be considered under the heading of gangliosidoses, although the complex disturbance of lipid and mucopolysaccharide metabolism in gargoylism is still poorly understood. 

Differential diagnosis from Tay-Sachs disease is possible on the basis of visceral storage, from Niemann-Pick disease and Gaucher s disease through the identification of the stored lipid and from Hurler s syndrome by the storage and excretion of acid mucopolysaccharides in the latter. 

The relationship between the increase of ganglioside content and storage of acid mucopolysaccharides is not known. Both groups of compounds share hexose... 

The storage polysaccharide in animals is glycogen. Structural functions are carried out by chitin, forming the exoskeletons of crustaceans, moUuscs and insects, and mucopolysaccharides occurring in proteoglycans of connective tissues. 

An inborn error of lipid storage characterized by the accumulation of ganglioside GMi. It is due to a deficiency of the enzyme fi-galactosidase which catalyses the removal of galactose from the GMi ganglioside molecule. The symptoms of the condition are somewhat similar to Hurler s syndrome but without the mucopolysaccharide excretion in the urine. 

Atherosclerotic plaque is a complex lesion which is a result of an inflammatory and reparative process. The atheroma plaque contains extracellular deposits of calcium salts, blood components, cholesterol crystals, and acid mucopolysaccharides. The initial changes, however, seem to occur at the cellular level, often accompanied by an abnormal intracellular storage of lipids, particularly cholesterol esters, fatty acids, and lipoprotein complexes. The rupture of the plaque can generate thrombosis, reductions in the vessel lumen and consequently in the cardiac perfusion, and acute or chronic consequences. 

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