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Pediatric Research

Volumn 49, Issue 5, 2001, Pages 627-635

Whole-body L-leucine oxidation in patients with variant form of maple syrup urine disease

(4) Schadewaldt, P a Bodner Leidecker, A a Hammen, H W a Wendel, U a

a Klinische Biochemie (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

2 OXOACID DEHYDROGENASE; 2 OXOISOCAPROIC ACID; BRANCHED CHAIN AMINO ACID; CARBON 13; LEUCINE;

ADOLESCENT; ADULT; ARTICLE; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; ENZYME ACTIVITY; FEMALE; HUMAN; MALE; MAPLE SYRUP URINE DISEASE; OXIDATION; PRIORITY JOURNAL;

EID: 0035035189 PISSN: 00313998 EISSN: None Source Type: Journal
DOI: 10.1203/00006450-200105000-00004 Document Type: Article

Times cited : (14)

References (32)

1
- 85037402886
- (2000)

2
- 0001106274
- Disorders of branched chain amino acid and keto acid metabolism
- Scriver CR, Beaudet AL, Sly WS, Valle D (eds). McGraw-Hill, New York
- (1995) The Metabolic and Molecular Bases of Inherited Disease , pp. 1239-1278
- Chuang, D.T.¹ Shih, V.E.²

3
- 0022249030
- So-called thiamin-responsive maple syrup urine disease: 15-year follow-up of the original patient
- (1985) J Pediatr , vol.107 , pp. 763-765
- Scriver, C.R.¹ Clow, C.L.² George, H.G.³

4
- 0023116657
- Effect of insulin on leucine kinetics in maple syrup urine disease
- (1987) Pediatr Res , vol.21 , pp. 10-13
- Collins, J.E.¹ Umpleby, A.M.² Boroujerdi, M.A.³ Leonard, J.V.⁴ Sonksen, P.H.⁵

5
- 0025267412
- Protein and leucine metabolism in maple syrup urine disease
- (1990) Am J Physiol , vol.258
- Thompson, G.N.¹ Bresson, J.L.² Pacy, P.J.³ Bonnefont, J.P.⁴ Walter, J.H.⁵ Leonard, J.V.⁶ Saudubray, J.M.⁷ Halliday, D.⁸

6
- 0025760904
- Acute illness in maple syrup urine disease: Dynamics of protein metabolism and implications for management
- (1991) J Pediatr , vol.119 , pp. 35-41
- Thompson, G.N.¹ Francis, D.E.M.² Halliday, D.³

7
- 0024989629
- In vivo enzyme activity in inborn errors of metabolism
- (1990) Metabolism , vol.39 , pp. 799-807
- Thompson, G.N.¹ Walter, J.H.² Leonard, J.V.³ Halliday, D.⁴

8
- 0027286639
- Practical method to estimate whole body leucine oxidation in maple syrup urine disease
- (1993) Pediatr Res , vol.33 , pp. 445-451
- Elsas II, L.J.¹ Ellerine, N.P.² Klein, P.D.³

9
- 31344482641
- Metabolism of branched-chain amino acids in maple syrup urine disease
- (1997) Eur J Pediatr , vol.156 , Issue.SUPPL. 1
- Schadewaldt, P.¹ Wendel, U.²

10
- 0031977378
- 13C]leucine breath tests: A reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects
- (1998) Pediatr Res , vol.43 , pp. 592-600
- Schadewaldt, P.¹ Bodner, A.² Brösicke, H.³ Hammen, H.-W.⁴ Wendel, U.⁵

11
- 0024439931
- Analysis of maple syrup urine disease in cell culture: Use of substrates
- (1989) Clin Chim Acta , vol.184 , pp. 47-56
- Schadewaldt, P.¹ Beck, K.² Wendel, U.³

12
- 0032857049
- Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease
- (1999) Clin Chem , vol.45 , pp. 1734-1740
- Schadewaldt, P.¹ Bodner-Leidecker, A.² Hammen, H.-W.³ Wendel, U.⁴

13
- 0000788257
- Branched-chain L-amino acid oxidation in overnight fasted human subjects
- (1997) Isotopes Environ Health Stud , vol.33 , pp. 189-196
- Bodner, A.¹ Hammen, H.-W.² Renn, W.³ Wendel, U.⁴ Schadewaldt, P.⁵

14
- 0003776456
- Wiley-Liss, New York
- (1992) Radioactive and Stable Isotope Tracers in Biomedicine: Principles and Practice of Kinetic Analysis , pp. 230-232
- Wolfe, R.R.¹

15
- 0022294449
- Use of reciprocal pool specific activity to model leucine metabolism in humans
- (1985) Am J Physiol , vol.249
- Schwenk, W.¹ Beaufrére, B.² Haymond, M.W.³

16
- 0030996515
- Protein requirements and ageing: Metabolic demand and efficiency of utilization
- (1997) Brit J Nutr , vol.77 , pp. 685-702
- Frereday, A.¹ Gibson, N.R.² Cox, M.³ Pacy, P.J.⁴ Millward, D.J.⁵

17
- 0024535295
- Intrinsically difficult problems: The kinetics of body proteins and amino acids in man
- (1989) Diabetes Metab Rev , vol.5 , pp. 111-132
- Bier, D.M.¹

18
- 0001760204
- Maple syrup urine disease, with particular reference to dietotherapy
- (1964) Pediatrics , vol.34 , pp. 454-462
- Snyderman, S.E.¹ Norton, P.M.² Roitman, E.³ Holt L.E., Jr.⁴

19
- 0020373407
- Maple syrup urine disease - Therapeutic use of insulin in catabolic states
- (1982) Eur J Pediatr , vol.139 , pp. 172-175
- Wendel, U.¹ Langenbeck, U.² Lombeck, I.³ Bremer, H.J.⁴

20
- 0005045320
- Acute and long-term treatment of children with maple syrup urine disease
- Adibi SA, Fekl W, Langenbeck U, Schauder P (eds). Karger, Basel
- (1984) Branched Chain Amino and Keto Acids in Health and Disease , pp. 335-347
- Wendel, U.¹

21
- 0021042770
- Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusion in maple syrup urine disease
- (1983) J Inherit Metab Dis , vol.6 , pp. 183-189
- Shigematsu, Y.¹ Kikuchi, K.² Momoi, T.³ Sudo, M.⁴ Kikawa, Y.⁵ Nosaka, K.⁶ Kuriyama, M.⁷ Haruki, S.⁸ Sanada, K.⁹ Hamano, M.¹⁰ Suzuki, Y.¹¹

22
- 0026074415
- Branched-chain amino acid free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease
- (1991) N Engl J Med , vol.324 , pp. 175-179
- Berry, G.T.¹ Heidenreich, R.² Kaplan, P.³ Levine, F.⁴ Mazur, A.⁵ Palmieri, M.J.⁶ Yudkoff, M.⁷ Segal, S.⁸

23
- 0028941849
- Effect of cortisol on energy expenditure and amino acid metabolism in humans
- (1995) Am J Physiol , vol.268
- Brillon, D.J.¹ Zheng, B.² Campbell, R.G.³ Matthews, D.⁴

24
- 0025946079
- Compartmental model of leucine kinetics in humans
- (1991) Am J Physiol , vol.262
- Cobelli, C.¹ Saccomani, M.O.² Tessari, P.³ Biolo, G.⁴ Luzi, L.⁵ Matthews, D.E.⁶

25
- 0002124664
- On the relation of leucine oxidation to total metabolic flux through the branched-chain 2-oxo acid dehydrogenase complex (BCOA-DH) in situ
- abstr
- (1998) J Inherit Metab Dis , vol.21 , Issue.SUPPL. 2 , pp. 18
- Schadewaldt, P.¹ Wendel, U.² Bodner, A.³

26
- 85037409437
- Thesis, Universität Düsseldorf, Düsseldorf, Germany
- (2000) Stoffwechsel verzweigtkettiger L-Aminosäuren bei Ahronsirupkrankheit , pp. 129-153
- Bodner-Leidecker, A.¹

27
- 0005066989
- Interactions among leucine, isoleucine and valine with special reference to branched-chain amino acid antagonism
- Friedman M (ed). CRC Press, Boca Raton, FL
- (1989) Absorption and Utilization of Amino Acids , pp. 229-244
- Block, K.P.¹

28
- 0031750339
- A molecular model of human branched-chain amino acid metabolism
- (1998) Am J Clin Nutr , vol.68 , pp. 72-81
- Suryawan, A.¹ Hawes, J.W.² Harris, R.A.³ Shimomura, Y.⁴ Jenkins, A.E.⁵ Hutson, S.M.⁶

29
- 0028817993
- Deamination of amino acids as source for ammonia production in human skeletal muscle during prolonged exercise
- (1995) J Physiol , vol.489 , pp. 251-261
- Van Hall, G.¹ Saltin, B.² Van der Vusse, G.J.³ Söderlund, K.⁴ Wagenmakers, A.J.M.⁵

30
- 0024461370
- Exercise-induced activation of the branched-chain 2-oxo acid dehydrogenase in human muscle
- (1989) Eur J Appl Physiol , vol.59 , pp. 159-167
- Wagenmakers, A.J.M.¹ Brookes, J.H.² Coakley, J.F.³ Reilly, T.⁴ Edwards, R.H.T.⁵

31
- 0030901554
- Nutritional status affects branched-chain oxoacid dehydrogenase activity during exercise in humans
- (1997) Am J Physiol , vol.272
- Jackman, M.L.¹ Gibala, M.J.² Hultman, E.³ Graham, T.E.⁴

32
- 0031795175
- Modulation of whole body protein metabolism, during and after exercise, by variation of dietary protein
- (1998) J Appl Physiol , vol.85 , pp. 1744-1752
- Bowtell, J.L.¹ Leese, G.P.² Smith, K.³ Watt, P.W.⁴ Nevill, A.⁵ Rooyackers, O.⁶ Wagenmakers, A.J.M.⁷ Rennie, M.J.⁸

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