Gargoylism

Gentle, Mary. Rats and gargoyles. London, New York Bantam P, 1990. 414 p ISBN 0-593-01948-2... 

You are too young to remember. You have been going around prying into those matters She ought not to scowl it made her as ugly as a gargoyle. 

Brante G (1952) Gargoylism a mucopolysaccharidosis. Scand J Clin Lab Invest 4 43-46... 

KUD-I a remote controlled glide bomb called Gargoyle... 

There are numerous inherited disorders of lysosomal metabolism in humans. These disorders result from the lack of a specific acid hydrolase and have several clinical manifestations. A variety of substances may accumulate that interfere with normal cell functions, as is the case with the lipidoses (Chapter 9) or mucopolysaccharides (glycosaminoglycans) in the Hurler s disease (gargoylism). 

Van Hoof F, Hers HG. L ultrastructure des cellules hepatiques dans la maladie de Hurler (gargoylism). CR. Acad. Sci. 1964 259 1281-1283. 

Brit desgn for SnCl used as sr producing agent a remote controlled glide bomb called Gargoyle symbol for signal pistol... 

The urine of patients with gargoylism, of both the autosomal recessive and sex-linked types, contains abnormally high amounts of acid mucopolysaccharides. Determination (T2) of the acid mucopolysaccharides in urine in relationship to preformed creatinine is useful in distinguishing certain cases of gargoylism from other conditions with which they are sometimes clinically confused (e.g., cretinism and chondro-osteo-dystrophies of the Morquio-Brailsford type). Another simple diagnostic test for gargoylism is based on the presence in the lymphocytes of abnormal inclusions of acid mucopolysaccharides which are revealed by staining (M24). 

Although the therapy of gargoylism is not well advanced, the discovery (Lll) of an apparently related heritable disorder of acid mucopolysaccharide metabolism in cattle may facilitate study of the pathogenesis and the therapy of this condition. 

M21. Meyer, K., Hoffman, P., Linker, A., Crumbach, M. M., and Sampson, F., Sulfated mucopolysaccharides of urine and organs in gargoylism (Hurler s syndrome) II. Additional studies. Proc. Soc. Exptl. Biol. Med. 102, 587-590 (1959). 

M24. Mittwoch, U., Inclusions of mucopolysaccharide in the lymphocytes of patients with gargoylism. Nature 191, 1315-1316 (1961). 

Austin, J. H., Some recent findings in leukodystrophies and in gargoylism. In Inborn Disorders of Sphingolipid Metabolism (S. N. Aronson and B. W. Volk, eds.), pp. 359-387. Pergamon, New York, 1967. 

W7. Wessler, E., Determination of acidic glycosaminoglycans (mucopolysaccharides) in urine by an ion exchange method. Application to coUagenoses , gargoylism, the nail-patella syndrome and Farber s disease. Clin. Chim. Acta 16,235-243 (1967). 

Gargoyles on the Notre Dame cathedral in Paris in need of restoration from decades of acid rain. [Witold Skrypczak/SuperStock)... 

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