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Compositional changes of urinary acidic glycosaminoglycans in progressive systemic sclerosis
Clinica Chimica Acta; International Journal of Clinical Chemistry
|November 20, 1980
Summary
Urinary acidic glycosaminoglycans (AGAG) are elevated in progressive systemic sclerosis (PSS). Heparan sulfates (HS) and specific chondroitin sulfate isomers increase, indicating abnormal AGAG metabolism in PSS patients.
Area of Science:
- Biochemistry
- Clinical Chemistry
- Rheumatology
Background:
- Acidic glycosaminoglycans (AGAG) are complex carbohydrates found in connective tissues.
- Alterations in AGAG metabolism are implicated in various connective tissue diseases.
- Progressive systemic sclerosis (PSS) is a systemic autoimmune disease affecting the skin and internal organs.
Purpose of the Study:
- To investigate the compositional changes of urinary acidic glycosaminoglycans (AGAG) in patients with progressive systemic sclerosis (PSS).
- To compare AGAG profiles in PSS patients with those of healthy individuals.
Main Methods:
- Enzyme assays using chondroitinases and heparitinase to analyze AGAG composition.
- Electrophoretic characterization of urinary AGAG.
- Paper chromatography for separating and quantifying unsaturated disaccharide units.
Main Results:
- Daily urinary excretion of AGAG was significantly increased in PSS patients compared to normal subjects.
- The proportion of heparan sulfates (HS) in urinary AGAG was significantly higher in PSS patients.
- An elevated ratio of 4-sulfated to total disaccharides was observed in the chondroitin sulfate fraction of PSS patients' urine.
Conclusions:
- Patients with PSS exhibit abnormal turnover of acidic glycosaminoglycans.
- Increased urinary heparan sulfates and altered chondroitin sulfate isomers suggest a specific metabolic derangement in PSS.
- Urinary AGAG analysis may serve as a potential biomarker for PSS.