[Non-enzymatic glycosylation of hemoglobin and serum protein in children with galactosemia].
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.
D000293:Adolescent; D001797:Blood Protein Electrophoresis; D001798:Blood Proteins / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D005693:Galactosemias / Q000097:blood*; D006442:Glycated Hemoglobin / Q000378:metabolism*; D006025:Glycosaminoglycans / Q000652:urine; D006441:Hemoglobin A / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008297:Male
D001798:Blood Proteins; D006442:Glycated Hemoglobin; D006025:Glycosaminoglycans; D006441:Hemoglobin A
Nichtenzymatische Glykosylierung von Hämoglobin und Serumprotein bei Kindern mit Galaktosämie.