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. 1972 Sep 1;136(3):644–649. doi: 10.1084/jem.136.3.644

THE HURLER SYNDROME: A STUDY OF CULTURED LYMPHOID CELL LINES

B Shannon Danes 1, T H Hütteroth 1, H Cleve 1, Alexander G Bearn 1
PMCID: PMC2139258  PMID: 4115134

Abstract

Lymphoid suspension lines have been established from three patients with the Hurler syndrome and four normals. The Hurler lines can be distinguished from normals by (a) staining characteristics, (b) increase in total cellular mucopolysaccharide content, and (c) increase in dermatan sulfate. Hyaluronic acid is absent in cultured lymphoid cells from normal persons and patients with the Hurler syndrome. The availability of biochemically marked suspension cultures should prove useful for enzymatic studies as well as for further elucidation of this clinical syndrome.

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Selected References

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  1. BITTER T., MUIR H. M. A modified uronic acid carbazole reaction. Anal Biochem. 1962 Oct;4:330–334. doi: 10.1016/0003-2697(62)90095-7. [DOI] [PubMed] [Google Scholar]
  2. BOWMAN J. E., MITTWOCH U., SCHNEIDERMAN L. J. Persistence of mucopolysaccharide inclusions in cultures of lymphocytes from patients with gargoylism. Nature. 1962 Aug 11;195:612–613. doi: 10.1038/195612a0. [DOI] [PubMed] [Google Scholar]
  3. CIFONELLI J. A., LUDOWIEG J., DORFMAN A. Chemistry of beta-heparin (chondroitinsulfuric acid-B). J Biol Chem. 1958 Sep;233(3):541–545. [PubMed] [Google Scholar]
  4. Danes B. S., Bearn A. G. Cystic fibrosis of the pancreas. A study in cell culture. J Exp Med. 1969 Apr 1;129(4):775–793. doi: 10.1084/jem.129.4.775. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Danes B. S., Bearn A. G. Hurler's syndrome. Effect of retinol (vitamin A alcohol) on cellular mucopolysaccharides in cultured human skin fibroblasts. J Exp Med. 1966 Dec 1;124(6):1181–1198. doi: 10.1084/jem.124.6.1181. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Danes B. S., Scott J. E., Bearn A. G. Further studies on metachromasia in cultured human fibroblasts. Staining of glycosaminoglycans (mucopolysaccharides) by Alcian blue in salt solutions. J Exp Med. 1970 Oct 1;132(4):765–774. doi: 10.1084/jem.132.4.765. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Di Ferrante N., Nichols B. L., Donnelly P. V., Neri G., Hrgovcic R., Berglund R. K. Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion. Proc Natl Acad Sci U S A. 1971 Feb;68(2):303–307. doi: 10.1073/pnas.68.2.303. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Foley K. M., Danes B. S., Bearn A. G. White blood cell cultures in genetic studies on the human mucopolysaccharidoses. Science. 1969 Apr 25;164(3878):424–426. doi: 10.1126/science.164.3878.424. [DOI] [PubMed] [Google Scholar]
  9. LAGUNOFF D., WARREN G. Determination of 2-deoxy-2-sulfoaminohexose content of mucopolysaccharides. Arch Biochem Biophys. 1962 Dec;99:396–400. doi: 10.1016/0003-9861(62)90285-0. [DOI] [PubMed] [Google Scholar]
  10. MITTWOCH U. Inclusions of mucopolysaccharide in the lymphocytes of patients with gargoylism. Nature. 1961 Sep 23;191:1315–1316. doi: 10.1038/1911315a0. [DOI] [PubMed] [Google Scholar]
  11. Matalon R., Cifonelli J. A., Dorfman A. L-iduronidase in cultured human fibroblasts and liver. Biochem Biophys Res Commun. 1971 Jan 22;42(2):340–345. doi: 10.1016/0006-291x(71)90108-2. [DOI] [PubMed] [Google Scholar]
  12. Matalon R., Dorfman A. Hurler's syndrome: biosynthesis of acid mucopolysaccharides in tissue culture. Proc Natl Acad Sci U S A. 1966 Oct;56(4):1310–1316. doi: 10.1073/pnas.56.4.1310. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. McKusick V. A. The nosology of the mucopolysaccharidoses. Am J Med. 1969 Nov;47(5):730–747. doi: 10.1016/0002-9343(69)90167-3. [DOI] [PubMed] [Google Scholar]
  14. Moore G. E., Ito E., Ulrich K., Sandberg A. A. Culture of human leukemia cells. Cancer. 1966 May;19(5):713–723. doi: 10.1002/1097-0142(196605)19:5<713::aid-cncr2820190518>3.0.co;2-y. [DOI] [PubMed] [Google Scholar]
  15. Wessler E. Analytical and preparative separation of acidic glycosaminoglycans by electrophoresis in barium acetate. Anal Biochem. 1968 Dec;26(3):439–444. doi: 10.1016/0003-2697(68)90205-4. [DOI] [PubMed] [Google Scholar]

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