par Grégoire, Philippe 
;Jonniaux, G. ;Voet, W.
Référence Clinica chimica acta, 33, 2, page (387-394)
Publication Publié, 1971-07
;Jonniaux, G. ;Voet, W.Référence Clinica chimica acta, 33, 2, page (387-394)
Publication Publié, 1971-07
Article révisé par les pairs
| Résumé : | We have studied glycolipids (ceramide-trihexosides and ceramide-dihexosides) extracted from urines of a case of Fabry's disease in a child. Ceramide-trihexosides are similar to those accumulated in liver and kidney. Ceramide-dihexosides contain sulfur and seem similar to the glycolipids isolated by Sweeley and Klionsky from the kidney of a patient suffering from Fabry's disease. However, in our urinary glycolipid, the sulfur is not in the sulfate form; it is not combined with the hexose but is located in the ceramide. About one atom corresponds to two molecules of sphingolipid. Our study of Fabry's disease has been extended to the fatty acid composition of urinary and hepatic glycolipids. None of the glycolipids studied contained hydroxylated fatty acids. The dihexoside contains more palmitic and less lignoceric acid than the trihexoside. © 1971. |
