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Biological Behavior of Lymphocytes in Hunter-Hurler's Disease

Biological Behavior of Lymphocytes in Hunter-Hurler's Disease Abstract HUNTER-HURLER'S disease is an born error of metabolism characterized by excessive deposition of acid mucopolysaccharides (chondroitin sulfate B and heparitin sulfate) in the liver, spleen, skin, and other organs whereas the nerve cells accumulate an abnormal amount of ganglioside containing lipids.1,2 The main clinical features consist of dwarfism, hepatosplenomegaly, bone and joint deformities, mental and physical deterioration, corneal clouding and abnormal and coarse facial features: bushy eyebrows, sunken and upturned nose, thick lips, and hirsutism. The onset is commonly between a few months and 5 years of age and these patients rarely survive past the age of adolescence. Dorfman and Lorincz3 and subsequently many others demonstrated that the affected children excrete an excessive amount of acid mucopolysaccharide (AMPS) in their urine, while Danes and Bearn4 and Matalon and Dorfman5 showed that skin fibroblasts from these patients grown in tissue culture accumulate a large quantity of References 1. Brante, G.: Gargoylism: A Mucopolysaccharidosis , Scand J Clin Lab Invest 4:43-46. 1952.Crossref 2. Meyer, K., et al: Sulfated Mucopolysaccharides of Urine and Organs in Gargoylism (Hurler's Syndrome): II. Additional Studies , Proc Soc Exper Biol Med 102:587-590 ( (Oct-Dec) ) 1959.Crossref 3. Dorfman, A., and Lorincz, A.E.: Occurrence of Urinary Acid Mucopolysaccharides in the Hurler's Syndrome , Proc Nat Acad Sci USA 43:443-446 ( (June 15) ) 1957.Crossref 4. Danes, B.S., and Beam, A.G.: Hurler's Syndrome: A Genetic Study in Cell Culture , J Exp Med 123:1-16 ( (Jan) ) 1965.Crossref 5. Metalon, R., and Dorfman, A.: Hurler's Syndrome: Biosynthesis of Acid Mucopolysaccharides in Tissue Culture , Proc Nat Acad Sci USA 56:1310-1316 ( (Oct) ) 1966.Crossref 6. Scheie, H.G.; Hambrick, G.W., Jr.; and Barness, L.A.: A Newly Recognized Forme Fruste of Hurler's Disease (Gargoylism) , Amer J Ophthal 53:753-769 ( (May) ) 1962. 7. Mittwoch, U.: Inclusions of Mucopolvsaccharides in the Lymphocytes of Patients With Gargoylism , Nature 191:1315-1316 ( (Sept 23) ) 1961.Crossref 8. Chessin, L.N., et al: Studies on Human Peripheral Blood Lymphocytes in Vitro: V. Biosynthesis of Immunoglobulins , J Immunol , to be published. 9. Rennert, O.M., and Dekaban, A.S.: Modification of Urinary Mucopolysaccharide Excretion in Patients With Hurler's Syndrome , Clin Pharmacol Ther 7:783-787 ( (Nov-Dec) ) 1966. 10. Bowman, J.E.; Mittwoch, U.; and Schneiderman, L.J.: Persistence of Mucopolysaccharide Inclusions in Cultures of Lymphocytes From Patients With Gargoylism , Nature 195:612-613 ( (Aug 11) ) 1962.Crossref 11. Reilly, W.A.: The Granules in the Leukocytes in Gargoylism , Amer J Dis Child 62:489-491 ( (Sept) ) 1941. 12. Gasser, C.: Contribution to discussion on A. Alder's article, Konstitutionell bedingte Granulationsveränderungen der Leukocyten und Knochenveränderungen , Schweiz Med Wschr 80:1095-1098 ( (Sept 30) ) 1950. 13. Muir, H.; Mittwoch, U.; and Bitter, T.: The Diagnostic Value of Isolated Urinary Mucopolysaccharides and of Lymphocyte Inclusions in Gargoylism , Arch Dis Child 38:358-363 ( (Aug) ) 1963.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Biological Behavior of Lymphocytes in Hunter-Hurler's Disease

Zelson, J.; Dekaban, A. S.
Archives of Neurology , Volume 20 (4) – Apr 1, 1969
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References (15)

Publisher
American Medical Association
Copyright
Copyright © 1969 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1969.00480100034004
Publisher site
See Article on Publisher Site

Abstract

Abstract HUNTER-HURLER'S disease is an born error of metabolism characterized by excessive deposition of acid mucopolysaccharides (chondroitin sulfate B and heparitin sulfate) in the liver, spleen, skin, and other organs whereas the nerve cells accumulate an abnormal amount of ganglioside containing lipids.1,2 The main clinical features consist of dwarfism, hepatosplenomegaly, bone and joint deformities, mental and physical deterioration, corneal clouding and abnormal and coarse facial features: bushy eyebrows, sunken and upturned nose, thick lips, and hirsutism. The onset is commonly between a few months and 5 years of age and these patients rarely survive past the age of adolescence. Dorfman and Lorincz3 and subsequently many others demonstrated that the affected children excrete an excessive amount of acid mucopolysaccharide (AMPS) in their urine, while Danes and Bearn4 and Matalon and Dorfman5 showed that skin fibroblasts from these patients grown in tissue culture accumulate a large quantity of References 1. Brante, G.: Gargoylism: A Mucopolysaccharidosis , Scand J Clin Lab Invest 4:43-46. 1952.Crossref 2. Meyer, K., et al: Sulfated Mucopolysaccharides of Urine and Organs in Gargoylism (Hurler's Syndrome): II. Additional Studies , Proc Soc Exper Biol Med 102:587-590 ( (Oct-Dec) ) 1959.Crossref 3. Dorfman, A., and Lorincz, A.E.: Occurrence of Urinary Acid Mucopolysaccharides in the Hurler's Syndrome , Proc Nat Acad Sci USA 43:443-446 ( (June 15) ) 1957.Crossref 4. Danes, B.S., and Beam, A.G.: Hurler's Syndrome: A Genetic Study in Cell Culture , J Exp Med 123:1-16 ( (Jan) ) 1965.Crossref 5. Metalon, R., and Dorfman, A.: Hurler's Syndrome: Biosynthesis of Acid Mucopolysaccharides in Tissue Culture , Proc Nat Acad Sci USA 56:1310-1316 ( (Oct) ) 1966.Crossref 6. Scheie, H.G.; Hambrick, G.W., Jr.; and Barness, L.A.: A Newly Recognized Forme Fruste of Hurler's Disease (Gargoylism) , Amer J Ophthal 53:753-769 ( (May) ) 1962. 7. Mittwoch, U.: Inclusions of Mucopolvsaccharides in the Lymphocytes of Patients With Gargoylism , Nature 191:1315-1316 ( (Sept 23) ) 1961.Crossref 8. Chessin, L.N., et al: Studies on Human Peripheral Blood Lymphocytes in Vitro: V. Biosynthesis of Immunoglobulins , J Immunol , to be published. 9. Rennert, O.M., and Dekaban, A.S.: Modification of Urinary Mucopolysaccharide Excretion in Patients With Hurler's Syndrome , Clin Pharmacol Ther 7:783-787 ( (Nov-Dec) ) 1966. 10. Bowman, J.E.; Mittwoch, U.; and Schneiderman, L.J.: Persistence of Mucopolysaccharide Inclusions in Cultures of Lymphocytes From Patients With Gargoylism , Nature 195:612-613 ( (Aug 11) ) 1962.Crossref 11. Reilly, W.A.: The Granules in the Leukocytes in Gargoylism , Amer J Dis Child 62:489-491 ( (Sept) ) 1941. 12. Gasser, C.: Contribution to discussion on A. Alder's article, Konstitutionell bedingte Granulationsveränderungen der Leukocyten und Knochenveränderungen , Schweiz Med Wschr 80:1095-1098 ( (Sept 30) ) 1950. 13. Muir, H.; Mittwoch, U.; and Bitter, T.: The Diagnostic Value of Isolated Urinary Mucopolysaccharides and of Lymphocyte Inclusions in Gargoylism , Arch Dis Child 38:358-363 ( (Aug) ) 1963.Crossref

Journal

Archives of Neurology – American Medical Association

Published: Apr 1, 1969

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