Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses

R. W. Burlingame, G. H. Thomas, R. L. Stevens, K. Schmid, H. W. Moser

Research output: Contribution to journalArticle

Abstract

Glycosaminoglycans in urine from patients representing the major different mucopolysaccharidoses were separated and measured by use of a procedure that requires only 2 mL of urine. The compounds were resolved by two-dimensional electrophoresis on cellulose acetate plates and made visible by staining with Alcian Blue. They were identified by co-migration with standard glycosaminoglycans, by digestion with specific glycosidases, and by specific degradation with HNO 2. They were quantitated by comparing the absorbance of eluates of the stained spots to appropriate standard curves for each glycosaminoglycan. This study revealed additional findings. About half of the patients excreted small amounts of heparin. Further, the keratan sulfate in samples from Morquio's disease patients migrated differently from authentic keratan sulfate unless digested with chondroitinase ABC. Our results for these diseases are in harmony with earlier reports.

Original languageEnglish (US)
Pages (from-to)124-128
Number of pages5
JournalClinical Chemistry
Volume27
Issue number1
StatePublished - 1981
Externally publishedYes

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Mucopolysaccharidoses
Glycosaminoglycans
Keratan Sulfate
Urine
Mucopolysaccharidosis IV
Chondroitin ABC Lyase
Cellulose Acetate Electrophoresis
Alcian Blue
Glycoside Hydrolases
Electrophoresis
Heparin
Digestion
Staining and Labeling
Degradation

ASJC Scopus subject areas

  • Clinical Biochemistry

Cite this

Burlingame, R. W., Thomas, G. H., Stevens, R. L., Schmid, K., & Moser, H. W. (1981). Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses. Clinical Chemistry, 27(1), 124-128.

Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses. / Burlingame, R. W.; Thomas, G. H.; Stevens, R. L.; Schmid, K.; Moser, H. W.

In: Clinical Chemistry, Vol. 27, No. 1, 1981, p. 124-128.

Research output: Contribution to journalArticle

Burlingame, RW, Thomas, GH, Stevens, RL, Schmid, K & Moser, HW 1981, 'Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses', Clinical Chemistry, vol. 27, no. 1, pp. 124-128.
Burlingame RW, Thomas GH, Stevens RL, Schmid K, Moser HW. Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses. Clinical Chemistry. 1981;27(1):124-128.
Burlingame, R. W. ; Thomas, G. H. ; Stevens, R. L. ; Schmid, K. ; Moser, H. W. / Direct quantitation of glycosaminoglycans in 2 mL of urine from patients with mucopolysaccharidoses. In: Clinical Chemistry. 1981 ; Vol. 27, No. 1. pp. 124-128.
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