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Items: 1 to 20 of 241

1.

Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.

Schadewaldt P, Hammen HW, Ott AC, Wendel U.

J Inherit Metab Dis. 1999 Aug;22(6):706-22.

PMID:
10472531
2.
3.

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.

Riazi R, Rafii M, Clarke JT, Wykes LJ, Ball RO, Pencharz PB.

Am J Physiol Endocrinol Metab. 2004 Jul;287(1):E142-9.

4.

Metabolism of branched-chain amino acids in maple syrup urine disease.

Schadewaldt P, Wendel U.

Eur J Pediatr. 1997 Aug;156 Suppl 1:S62-6. Review.

PMID:
9266218
6.

Effect of the branched-chain alpha-keto acids accumulating in maple syrup urine disease on S100B release from glial cells.

Funchal C, Tramontina F, Quincozes dos Santos A, Fraga de Souza D, Gonçalves CA, Pessoa-Pureur R, Wajner M.

J Neurol Sci. 2007 Sep 15;260(1-2):87-94.

PMID:
17499767
7.

Transamination and oxidative decarboxylation rates of branched-chain 2-oxo acids in cultured human skin fibroblasts.

Schadewaldt P, Radeck W, Hammen HW, Wendel U.

Pediatr Res. 1988 Jan;23(1):40-4.

PMID:
3340442
8.

Thiamine response in maple syrup urine disease.

Fernhoff PM, Lubitz D, Danner DJ, Dembure PP, Schwartz HP, Hillman R, Bier DM, Elsas LJ.

Pediatr Res. 1985 Oct;19(10):1011-6.

PMID:
3903643
9.

Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.

Funchal C, Latini A, Jacques-Silva MC, Dos Santos AQ, Buzin L, Gottfried C, Wajner M, Pessoa-Pureur R.

Neurochem Int. 2006 Dec;49(7):640-50.

PMID:
16822590
10.

Oral L-alloisoleucine loading studies in healthy subjects and in patients with maple syrup urine disease.

Schadewaldt P, Dalle-Feste C, Langenbeck U, Wendel U.

Pediatr Res. 1991 Nov;30(5):430-4.

PMID:
1754297
11.

Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.

Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B.

Int J Neurosci. 1994 Nov;79(1-2):21-45.

PMID:
7744549
12.

Antioxidant administration prevents memory impairment in an animal model of maple syrup urine disease.

Scaini G, Teodorak BP, Jeremias IC, Morais MO, Mina F, Dominguini D, Pescador B, Comim CM, Schuck PF, Ferreira GC, Quevedo J, Streck EL.

Behav Brain Res. 2012 May 16;231(1):92-6. doi: 10.1016/j.bbr.2012.03.004.

PMID:
22433584
13.

Diagnosis and treatment of maple syrup disease: a study of 36 patients.

Morton DH, Strauss KA, Robinson DL, Puffenberger EG, Kelley RI.

Pediatrics. 2002 Jun;109(6):999-1008.

PMID:
12042535
14.

[Clinical characteristics and analysis of mass spectrometric data in 33 patients with maple syrup urine disease].

Yang N, Han LS, Ye J, Qiu WJ, Zhang HW, Gao XL, Wang Y, Li XY, Xu H, Gu XF.

Zhonghua Yi Xue Za Zhi. 2012 Oct 30;92(40):2839-42. Chinese.

PMID:
23290213
15.

Diurnal changes in plasma amino acids in maple syrup urine disease.

Schwahn B, Wendel U, Schadewaldt P, Falkenberg N, Mönch E.

Acta Paediatr. 1998 Dec;87(12):1245-6.

PMID:
9894823
17.
18.

Determination of R- and S-3-methyl-2-oxopentanoate enantiomers in human plasma: suitable method for label enrichment analysis.

Schadewaldt P, Wendel U, Hammen HW.

J Chromatogr B Biomed Appl. 1996 Jul 12;682(2):209-18.

PMID:
8844412
19.
20.

Diagnosis of MSUD by newborn screening allows early intervention without extraneous detoxification.

Heldt K, Schwahn B, Marquardt I, Grotzke M, Wendel U.

Mol Genet Metab. 2005 Apr;84(4):313-6.

PMID:
15781191
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