Total branched-chain amino acids requirement in patients with maple syrup urine disease by use of indicator amino acid oxidation with L-[1- 13C]phenylalanine

American Journal of Physiology - Endocrinology and Metabolism

Volumn 287, Issue 1 50-1, 2004, Pages

  Riazi, Roya ^a,b   Rafii, Mahroukh ^b   Clarke, Joe T R ^a,b   Wykes, Linda J ^c   Ball, Ronald O ^a,d   Pencharz, Paul B ^a,b,d,e  

^a UNIVERSITY OF TORONTO   (Canada)

^b HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^c MCGILL UNIVERSITY   (Canada)

^d UNIVERSITY OF ALBERTA   (Canada)

^e HOSPITAL FOR SICK CHILDREN   (Canada)

Author keywords

Amino acid requirements; Indicator amino acid oxidation

Indexed keywords

2 OXOACID DEHYDROGENASE; BRANCHED CHAIN AMINO ACID; CARBON 13; CARBON DIOXIDE; EGG PROTEIN; ISOLEUCINE; LEUCINE; PHENYLALANINE; VALINE;

ADULT; AMINO ACID METABOLISM; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; CLINICAL ARTICLE; CONCENTRATION (PARAMETERS); DECARBOXYLATION; ENZYME DEFICIENCY; FEMALE; HUMAN; MALE; MAPLE SYRUP URINE DISEASE; NUTRITION; OXIDATION; PRIORITY JOURNAL;

ADOLESCENT; ADULT; AMINO ACIDS, BRANCHED-CHAIN; AMINO ACIDS, ESSENTIAL; ENERGY METABOLISM; FEMALE; HUMANS; MALE; MAPLE SYRUP URINE DISEASE; METABOLIC CLEARANCE RATE; NUTRITIONAL REQUIREMENTS; OXIDATION-REDUCTION; PHENYLALANINE; RADIOISOTOPE DILUTION TECHNIQUE;

EID: 3042697192     PISSN: 01931849     EISSN: None     Source Type: Journal    
DOI: 10.1152/ajpendo.00431.2003     Document Type: Article

References (40)

1. Azcue M. Resting Energy Expenditure and Body Composition in Normal and Sick Children. Toronto, Canada: University of Toronto, 1992.
2. Bell L, Jones PLH, Telch J, Clandinin MT, and Pencharz PB. Prediction of energy needs for clinical trials. Nutr Res 5: 123-129, 1985.
3. Bidlingmeyer BA, Cohen SA, and Tarvin TL. Rapid analysis of amino acids using precolumn derivatization. J Chromatogr 336: 93-104, 1984.
4. Bross R, Ball RO, Clarke JTR, and Pencharz PB. Tyrosine requirements in children with classical phenylketonuria determined by indicator amino acid oxidation. Am J Physiol Endocrinol Metab 278: E195-E201, 2000.
5. Bross R, Ball RO, and Pencharz PB. Development of a minimally invasive protocol for determination of phenylalanine and lysine kinetics in humans during the fed state. J Nutr 128: 1913-1919, 1998.
6. Champe PC and Harvey RA. Amino acids: metabolism of carbon atoms. In: Biochemistry, edited by Champe PC and Harvey RA. Philadelphia, PA: Lippincott, 1987, p. 242-251.
7. Chuang DT and Shih VE. Disorders of branched chain amino acid and keto acid metabolism. In: The Metabolic and Molecular Basis of Inherited Disease (7th ed.), edited by Scriver CR, Baudet AL, Sly WS, and Valle D. New York: McGraw-Hill, 1995, p. 1239-1277.
8. Cohen SA, Bidlingmeyer BA, and Tarvin TL. PITC derivatives in amino acid analysis. Nature 320: 769-770, 1986.
9. Cohen SA and Strydom DJ. Amino acid analysis utilizing phenylisothiocyanate derivatives. Anal Biochem 174: 1-16, 1988.
10. Courtney-Martin G, Bross R, Rafii M, Clarke JTR, Ball RO, and Pencharz PB. Phenylalanine requirement in children with classical PKU determined by indicator amino acid oxidation. Am J Physiol Endocrinol Metab 283: E1249-E1256, 2002.
11. Danner DJ and Elsas LJ II. Disorders of branched chain amino acid and ketoacid metabolism. In: The Metabolic Basis of Inherited Disease, edited by Scriver CR, Baudet AL, Sly WS, and Valle D. New York: McGraw-Hill, 1989, p. 671-693.
12. Durnin JVGA and Rahaman MM. The assessment of the amount of fat in the human body from measurement of skinfold thickness. Br J Nutr 21: 681-689, 1967.
13. 15N]urea. Am J Clin Nutr 59: 1000-1011, 1994.
14. Food and Nutrition Board, National Research Council. Dietary Reference Intakes for Energy, Carbohydrate, Fibre, Fat, Fatty Acids, Cholesterol, Protein and Amino Acid. Institute of Medicine of National Academies. Washington, DC: National Academy Press, 2002.
15. Francis DEM. Dietary management of disorders of amino acid metabolism, organic acidemias, and urea cycle defects. In: Diets for Sick Children (4th ed.), edited by Francis DEM. Boston, MA: Blackwell Scientific, 1987, p. 263-313.
16. Fuller MF and Garlick PJ. Human amino acid requirements: can controversy be resolved? Annu Rev Nutr 14: 217-241, 1994.
17. Harper AE, Miller RH, and Block KP. Branched chain amino acid metabolism. Ann Rev Nutr 4: 409-454, 1984.
18. 2 infused by gut and vein: effect of feeding. Am J Physiol Endocrinol Metab 257: E426-E438, 1989.
19. Horber FF, Horbe-Feyder CM, Krayer S, Schwenk WF, and Haymond MW. Plasma reciprocal pool specific activity predicts that of intracellular free leucine for protein synthesis. Am J Physiol Endocrinol Metab 257: E385-E399, 1989.
20. 13C]phenylalanine. Am J Clin Nutr 68: 303-310, 1998.
21. Lukaski HC, Bolonchuk WW, Hall CB, and Siders WA. Validation of tetrapolar bioelectrical impedance method to assess body composition. J Appl Physiol 60: 1327-1332, 1986.
22. 13C]leucine. Am J Physiol Endocrinol Metab 238: E473-E479, 1980.
23. McGilvery RW and Goldstein GW. Amino acids: disposal of the carbon skeleton. In: Biochemistry: a Functional Approach, edited by McGilvery RW and Goldstein GW. Philadelphia, PA: Saunders, 1983, p. 593-617.
24. Menkes JH, Hurst PL, and Craig JM. A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance. Pediatrics 14: 462-466, 1954.
25. Patterson BW, Hatchey DL, Cook GL, Amann JM, and Klein PD. Incorporation of a stable isotopically labelled amino acid into multiple human apolipoprotein. J Lipid Res 32: 1063-1072, 1991.
26. Pencharz PB and Azcue M. Use of bioelectrical impedance analysis measurements in the clinical management of malnutrition. Am J Clin Nutr 64, Suppl: 485S-488S, 1996.
27. 13C]phenylalanine. J Nutr 133: 1383-1389, 2003.
28. Schadewaldt P and Wendel U. Metabolism of branched chain amino acids in maple syrup urine disease. Eur J Pediatr 156: S62-S66, 1997.
29. Seber GAF. Linear Regression Analysis. New York: Wiley, 1977.
30. Siri WE. Body composition from fluid spaces and density: analysis of methods. 1961. Nutrition 9: 480-491, 1993.
31. Trahms CM. Nutritional care in metabolic disorders. In: Krause's Food, Nutrition and Diet Therapy, edited by Mahan KL and Arlin M. Philadelphia, PA: Saunders, 1992, p. 699-716.
32. Wykes LJ, Ball RO, Menendez CE, and Pencharz PB. Urine collection as an alternative to blood sampling: a noninvasive means of determining isotope enrichment to study amino acid flux in neonates. Eur J Clin Nutr 44: 605-608, 1990.
33. Young VR. Protein and amino acids. In: Present Knowledge in Nutrition (8th ed.), edited by Bowman BA and Russel RM. Washington, DC: ILSI Press, 2001, p. 43-58.
34. Young VR, Bier DM, and Pellet PL. A theoretical basis for increasing current estimates of the amino acid requirements in adult man with experimental support. Am J Clin Nutr 50: 80-92, 1989.
35. 5]phenylalanine in humans: evidence for an isotope effect in renal tubular reabsorption. Metabolism 43: 487-491, 1994.
36. Zello GA, Pencharz PB, and Ball RO. The design and validation of a diet for studies of amino acid metabolism in adult humans. Nutr Res 10: 1353-1365, 1990.
37. 13C]phenylalanine. Am J Physiol Endocrinol Metab 259: E835-E843, 1990.
38. 13C]phenylalanine. Am J Physiol Endocrinol Metab 264: E677-E685, 1993.
39. Zello GA, Telch J, Clarke R, Ball RO, and Pencharz PB. Reexamination of protein requirements in adult male humans by end-product measurements of leucine and lysine metabolism. J Nutr 122: 1000-1008, 1992.
40. Zello GA, Wykes LJ, Ball RO, and Pencharz PB. Recent advances in methods of assessing dietary amino acid requirements for adult humans. J Nutr 125: 2907-2915, 1995.