[Non-enzymatic glycosylation of hemoglobin and serum protein in children with galactosemia].

Abstract

Non-enzymatic galactosylation has been investigated by in vitro incubation of red cell haemolysate, a HbAo-preparation and of GBM of healthy children. The effects of non-enzymatic galactosylation of haemoglobin has been studied by high pressure liquid chromatography, the effects of GBM galactosylation by immunoelectrophoresis. Subsequently, the occurrence of elevated values for HbAIa-c and GSP was evaluated in 14 galactosaemic children (11 transferase deficiency, 3 galactokinase deficiency), as well as urinary acid glycosaminoglycae excretion and GBM immunoelectrophoretic mobility in 6 of these 14 children measured. The results were compared to the respective values of healthy control children. After exclusion of significant non-enzymatic glucosylation by measuring postprandial blood glucose values the galactosaemic children showed significantly increased values for HbAIa-c (8.85 +/- 2.0% versus 7.7 +/- 0.3%; p less than 0.02), for GSP (0.43 +/- 0.13 mmol 5-HMF/mg protein versus 0.32 +/- 0.07 mmol 5-HMF/mg protein; p less than 0.005) as well as for urinary acid glycosaminoglycane excretion (45.3 +/- 23.4 micrograms/mg kreatinine versus 9.9 +/- 2.3 micrograms/mg Kreatinine; p less than 0.01). 3 out of the 6 children showed alpha 1-immunoelectrophoretic mobility of GBM antigens which was found also after incubation of GMB with galactose. The other 3 children had alpha 2-immobility, which was found in the healthy controls as well as in the control incubations. The impact of galactose on increased non-enzymatic glycosylation in children with galactosaemia as well as the significance of this finding for diagnostic purposes or for clarifying pathophysiological aspects of the disease remains to be studied further.

• Authors: H Coradello, A Pollak, S Scheibenreiter, E Legenstein, S Levin, G Lubec
• Journal: Wiener klinische Wochenschrift (Wien Klin Wochenschr) Vol. 95 Issue 22 Pg. 804-9 (Nov 25 1983) ISSN: 0043-5325 [Print] Austria
• Vernacular Title: Nichtenzymatische Glykosylierung von Hämoglobin und Serumprotein bei Kindern mit Galaktosämie.
• PMID: 6670278 (Publication Type: Journal Article)

Chemical References

• Blood Proteins
• Glycated Hemoglobin A
• Glycosaminoglycans
• Hemoglobin A

Topics

• Adolescent
• Blood Protein Electrophoresis
• Blood Proteins (metabolism)
• Child
• Child, Preschool
• Chromatography, High Pressure Liquid
• Erythrocytes (metabolism)
• Female
• Galactosemias (blood)
• Glycated Hemoglobin (metabolism)
• Glycosaminoglycans (urine)
• Hemoglobin A (metabolism)
• Humans
• Infant
• Male