Erythrocytic uridine diphosphate galactose in galactosaemia.
An earlier claim of a deficiency of uridine diphosphate galactose in erythrocytes of galactosaemia patients was not confirmed. Enzymic techniques similar to those of the earlier investigators were used to determine not only the concentration of uridine diphosphate galactose but also the ratio of this concentration to the sum of the uridine sugar diphosphates (uridine diphosphate galactose and uridine diphosphate glucose). The values in erythrocytes of galactosaemic subjects were similar to those of non-galactosaemic children on a galactose-restricted diet and to those of normal adults. These results cast doubt on the claim of a major deficiency of uridine diphosphate galactose in galactosaemia and on the need for treating galactosaemic children with uridine.
Kirkman
H N
HN
Brain and Development Research Center, University of North Carolina School of Medicine, Chapel Hill 27599-7250.
D000328:Adult; D001774:Blood Chemical Analysis; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D005693:Galactosemias / Q000097:blood* / Q000188:drug therapy; D006801:Humans; D008297:Male; D009243:NAD; D014529:Uridine / Q000627:therapeutic use; D014531:Uridine Diphosphate Galactose / Q000097:blood* / Q000172:deficiency; D014532:Uridine Diphosphate Glucose / Q000097:blood
D009243:NAD; D014531:Uridine Diphosphate Galactose; D014532:Uridine Diphosphate Glucose; D014529:Uridine