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Results: 1 to 20 of 166

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
[PubMed - indexed for MEDLINE]
2.

On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease.

Schadewaldt P, Hammen HW, Dalle-Feste C, Wendel U.

J Inherit Metab Dis. 1990;13(2):137-50.

PMID:
2116545
[PubMed - indexed for MEDLINE]
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. Epub 2004 Feb 17.

PMID:
14970005
[PubMed - indexed for MEDLINE]
Free Article
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
[PubMed - indexed for MEDLINE]
6.

Functional differences in the catabolism of branched-chain L-amino acids in cultured normal and maple syrup urine disease fibroblasts.

Schadewaldt P, Wendel U.

Biochem Med Metab Biol. 1989 Apr;41(2):105-16.

PMID:
2719855
[PubMed - indexed for MEDLINE]
7.

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. Epub 2007 May 17.

PMID:
17499767
[PubMed - indexed for MEDLINE]
8.

Determination of branched chain amino acids, methionine, phenylalanine, tyrosine and alpha-keto acids in plasma and dried blood samples using HPLC with fluorescence detection.

Kand'ár R, Záková P, Jirosová J, Sladká M.

Clin Chem Lab Med. 2009;47(5):565-72. doi: 10.1515/CCLM.2009.123.

PMID:
19290779
[PubMed - indexed for MEDLINE]
9.

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
[PubMed - indexed for MEDLINE]
10.

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
[PubMed - indexed for MEDLINE]
11.

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. Epub 2006 Jul 5.

PMID:
16822590
[PubMed - indexed for MEDLINE]
12.

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
[PubMed - indexed for MEDLINE]
13.

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
[PubMed - indexed for MEDLINE]
14.

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. Epub 2012 Mar 13.

PMID:
22433584
[PubMed - indexed for MEDLINE]
15.

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
[PubMed - indexed for MEDLINE]
16.

[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
[PubMed - indexed for MEDLINE]
17.

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
[PubMed - indexed for MEDLINE]
18.

Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination.

Treacy E, Clow CL, Reade TR, Chitayat D, Mamer OA, Scriver CR.

J Inherit Metab Dis. 1992;15(1):121-35.

PMID:
1583867
[PubMed - indexed for MEDLINE]
19.

Maple Syrup Urine Disease.

Strauss KA, Puffenberger EG, Morton DH.

In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, Fong CT, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2014.
2006 Jan 30 [updated 2013 May 09].

PMID:
20301495
[PubMed]
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