Elevation of erythrocyte redox potential linked to galactonate biosynthesis: Elimination by tolrestat

Metabolism: Clinical and Experimental

Volumn 47, Issue 11, 1998, Pages 1423-1428

  Berry, G T ^a   Wehrli, S ^b   Reynolds, R ^b   Palmieri, M ^b   Frangos, M ^b   Williamson, J R ^b   Segal, S ^b  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

GALACTITOL; GALACTONIC ACID; GALACTOSE 1 PHOSPHATE URIDYLYLTRANSFERASE; GALACTOSE PHOSPHATE; HEXOSE; LACTIC ACID; PYRUVIC ACID; TOLRESTAT; UNCLASSIFIED DRUG;

ADULT; ARTICLE; BIOACCUMULATION; CARBOHYDRATE METABOLISM; CASE REPORT; CONTROLLED STUDY; DRUG EFFECT; DRUG MECHANISM; ENZYME ACTIVITY; ENZYME DEFICIENCY; ERYTHROCYTE METABOLISM; GALACTOSEMIA; HEXOSE MONOPHOSPHATE SHUNT; HUMAN; HUMAN CELL; OXIDATION REDUCTION POTENTIAL; PRIORITY JOURNAL; TIME;

EID: 0031786754     PISSN: 00260495     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0026-0495(98)90317-1     Document Type: Article

References (27)

1. Segal S, Berry GT: Disorders of galactose metabolism, in Scriver CR, Beaudet AL, Sly WS, Valle D (eds): The Metabolic and Molecular Bases of Inherited Disease. New York, NY, McGraw-Hill, 1995, pp 967-1000
2. Waggoner DD, Buist NR, Donnell GN: Long-term prognosis in galactosaemia: Results of a survey of 350 cases. J Inherit Metab Dis 13:802-818, 1990
3. Berry GT, Nissim I, Lin Z, et al: Endogenous synthesis of galactose in normal man and patients with hereditary galactosemia. Lancet 346:1073-1074, 1995
4. Schwarz V: The value of galactose phosphate determinations in the treatment of galactosaemia. Arch Dis Child 35:428-432, 1960
5. Jakobs C, Schweitzer S, Dorland B: Galactitol in galactosemia. Eur J Pediatr 154:S50-S52, 1995 (suppl)
6. Wehrli S, Berry GT, Palmieri MJ, et al: Urinary galactonate in patients with galactosemia: Quantitation by NMR spectroscopy. Pediatr Res 42:855-861, 1997
7. Wermuth B: Aldo-keto reductases, in Flynn TG, Wermuth B (eds): Enzymology of Carbonyl Metabolism 2: Aldehyde Dehydrogenase, Aldo-Keto Reductase and Alcohol Dehydrogenase. New York, NY, Liss, 1985, pp 209-230
8. Berry GT: The role of polyols in the pathophysiology of galactosemia. Eur J Pediatr 154:S53-S64, 1995 (suppl)
9. 14C]galactitol in mammalian tissue. Biochim Biophys Acta 156:9-16, 1968
10. Berry GT, Palmieri M, 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 16:91-100, 1993
11. Cuatrecasas P, Segal S: Galactose conversion to D-xylulose: An alternate route of galactose metabolism. Science 153:549-551, 1966
12. Cuatrecasas P, Segal S: Mammalian galactose dehydrogenase. I. Identification and purification in rat liver. J Biol Chem 241:5904-5909, 1966
13. Rancour NJ, Hawkins ED, Wells WW: Galactose oxidation in liver. Arch Biochem Biophys 193:232-241, 1979
14. Quan-Ma R, Wells HJ, Wells WW, et al: Galactitol in the tissues of a galactosemic child. Am J Dis Child 112:477-478, 1966
15. Elsas LJ, Langley S, Paulk EM, et al: A molecular approach to galactosemia. Eur J Pediatr 154:S21-S27, 1995 (suppl)
16. Lai K, Langley SD, Singh R, et al: Identification of a mutation prevalent among African-Americans. J Pediatr 128:88-95, 1996
17. Berry GT, Palmieri MJ, Heales S, et al: Red blood cell uridine sugar nucleotide levels in patients with classic galactosemia. Metabolism 41:783-787, 1992
18. Bergmeyer HU: Pyruvate and L-lactate, in Methods of Enzymatic Analysis, vol 6 (ed 3). Weinheim, Germany, Verlag Chemie, 1984
19. Schwarz V, Goldberg L, Komrower GM, et al: Some disturbances of erythrocyte metabolism in galactosaemia. Biochem J 62:34-40, 1956
20. Kalckar HM, Anderson EP, Isselbacher KJ: Galactosemia, a congenital defect in a nucleotide transferase. Biochim Biophys Acta 20:262-268, 1956
21. Rogers S, Lichtenstein G, Gentile, et al: Accumulation of galactonate in liver of suckling rats perfused with galactose. Biochem Biophys Res Commun 118:304-309, 1984
22. 14C galactose by patients with congenital galactosemia: Evidence for a direct oxidative pathway. Am J Med 44:340-347, 1968
23. Bergren WR, Ng WG, Donnell GN, et al: Galactonic acid in galactosemia: Identification in the urine. Science 176:683-684, 1972
24. Airey CM, Price DE, Kemp JV, et al: The effect of aldose reductase inhibition on erythrocyte polyols and galactitol accumulation in diabetic patients. Diabet Med 6:804-808, 1989
25. Williamson JR, Chang K, Frangos M, et al: Hyperglycemic pseudohypoxia and diabetic complications. Diabetes 42:801-813, 1993
26. Travis SF, Morrison AD, Clements RS Jr, et al: Metabolic alterations in the human erythrocyte produced by increases in glucose concentration. J Clin Invest 50:2104-2112, 1971
27. Maxwell ES: The enzymatic interconversion of uridine diphosphogalactose and uridine diphosphoglucose. J Biol Chem 229:139-151, 1957