Sugar nucleotide concentrations in red blood cells of patients on protein- and lactose-limited diets: Effect of galactose supplementation

American Journal of Clinical Nutrition

Volumn 63, Issue 5, 1996, Pages 704-708

  Gibson, James B ^a   Berry, Gerard T ^b   Palmieri, Michael J ^b   Reynolds, Robert A ^b   Mazur, Alice T ^b   Segal, Stanton ^b  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

^b NONE

Author keywords

Galactose; maple syrup urine disease; uridine diphosphate galactose

Indexed keywords

GALACTOSE; SUGAR NUCLEOTIDE; URIDINE DIPHOSPHATE GALACTOSE; URIDINE DIPHOSPHATE GLUCOSE 4 EPIMERASE;

ARTICLE; CARBOHYDRATE INTAKE; CHILD; DIET RESTRICTION; DIET SUPPLEMENTATION; DIET THERAPY; FOOD COMPOSITION; HUMAN; INBORN ERROR OF METABOLISM; MAJOR CLINICAL STUDY; MAPLE SYRUP URINE DISEASE; PHENYLKETONURIA; PROTEIN RESTRICTION;

EID: 0029939296     PISSN: 00029165     EISSN: None     Source Type: Journal    
DOI: 10.1093/ajcn/63.5.704     Document Type: Article

References (15)

1. Maxwell ES. The enzymatic interconversion of uridme diphosphogalactose and uridine diphosphoglucose. J Biol Chem 1957;229:139-51.
2. Ng WG, Xu YK, Kaufman FR, Donnell GN. Deficit of uridine diphosphate galactose in galactosemia. J Inherit Metab Dis 1989;12: 257-66.
3. Berry GT, Palmieri MJ, Heales S, Leonard JV, Segal S. Red blood cell undine sugar nucleotide levels in patients with classic galactosemia and other metabolic disorders. Metabolism 1992;41:783-7.
4. Keevill NJ, Holton JB, Allen JT. The investigation of UDPglucose and UDPgalactose concentration in red blood cells of patients with classical galactosemia. Clin Chim Acta 1993;221:135-42.
5. Gibson JB, Reynolds RA, Palmieri MJ, et al. Comparison of erythrocyte uridine sugar nucleotide levels in normals, classic galactosemics and patients with other metabolic disorders. Metabolism 1995;44:597-604.
6. Gibson JB, Berry GT, Mazur A, Palmieri MJ, Reynolds RA, Segal S. Effect of glucose and galactose loading in normal subjects on red and white blood cell uridine diphosphate sugars. Biochem Mol Med 1995; 55:8-14.
7. Posati LP, Orr ML. Composition of foods: dairy and egg products. Agriculture handbook no. 8-1. Washington, DC: US Department of Agriculture Agricultural Research Service, 1976.
8. Gross KC, Acosta PB. Fruits and vegetables are a source of galactose. implications in planning the diets of patients with galactosaemia J Inherit Metab Dis 1991;14:253-8.
9. Cropper SS, Gross KC, Olds SJ. Galactose content of selected fruit and vegetable baby foods: implications for infants on galactose-restricted diets. J Am Diet Assoc 1993;93:328-30.
10. Palmieri MJ, Berry GT, Player DA, Rogers S, Segal S. The concentration of red blood cell UDPglucose and UDPgalactose determined by HPLC. Anal Biochem 1991;194:388-93.
11. Palmieri MJ, Reynolds RA, Gibson JB, Berry GT, Segal S. The concentration of white blood cell UDPgalactose and UDPglucose determined by high performance liquid chromatography. Enzyme Protein 1993;47:105-15.
12. Berry GT, Palmieri MJ, Gross KC, et al. The effects of dietary fruits and vegetables on urinary galactitol excretion in galactose-1-phosphate uridyltransferase deficiency. J Inherit Metab Dis 1993;16:91-100.
13. Kirkman HN. Erythrocyte uridine diphosphate galactose in galactosemia. J Inherit Metab Dis 1992;15:4-16.
14. Cortes P, Dumler F, Paielle DL, Levin NW. Glomerular uracil nucleotide synthesis: effects of diabetes and protein intake. Am J Physiol 1988;255:F647-55.
15. McDowell MA, Biefel RR, Alaimo K, et al. Energy and macronutrient intakes of persons ages 2 months and over in the United States: third National Health and Nutrition Examination Survey. 1994;1:1988-91 (Advance data NCHS 255.)