Glycoasparagine metabolites in patients with aspartylglycosaminuria: comparison between english and finnish patients with special reference to storage materials
1973; Elsevier BV; Volume: 47; Issue: 1 Linguagem: Inglês
10.1016/0009-8981(73)90061-2
ISSN1873-3492
AutoresJ. Palo, R. J. Pollitt, Kathleen M. Pretty, H. Savolainen,
Tópico(s)Alcohol Consumption and Health Effects
ResumoUrine from Finnish patients with aspartylglycosaminuria has the same pattern of glycoasparagines as that previously described in English patients, and urine from the English patients contains the acidic carbohydrate detected in the Finnish patients' urine. Comparison with purified glycoasparagines indicates that the urinary acidic carbohydrate detected on thin-layer chromatography can be accounted for in terms of some of the higher glycoasparagines. Quantitative examination of extracts of post mortem brain and liver from an aspartylglycosaminuria patient shows a similar situation, but the higher glycoasparagines are present only as minor components. The only quantitatively important storage material yet identified in these organs is 2-acetamido-1-(β'-l-aspartamido)-1,2-dideoxy-β-d-glucose itself.
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