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Results: 20

1.

Leucine toxicity in a neuronal cell model with inhibited branched chain amino acid catabolism.

Kasinski A, Doering CB, Danner DJ.

Brain Res Mol Brain Res. 2004 Mar 30;122(2):180-7.

PMID:
15010210
[PubMed - indexed for MEDLINE]
3.

Human mutations affecting branched chain alpha-ketoacid dehydrogenase.

Danner DJ, Doering CB.

Front Biosci. 1998 Jun 3;3:d517-24. Review.

PMID:
9609836
[PubMed - indexed for MEDLINE]
4.

Maple syrup urine disease: it has come a long way.

Chuang DT.

J Pediatr. 1998 Mar;132(3 Pt 2):S17-23. Review.

PMID:
9546032
[PubMed - indexed for MEDLINE]
5.

Lessons from genetic disorders of branched-chain amino acid metabolism.

Chuang DT, Chuang JL, Wynn RM.

J Nutr. 2006 Jan;136(1 Suppl):243S-9S. Review.

PMID:
16365091
[PubMed - indexed for MEDLINE]
Free Article
6.

Animal models of maple syrup urine disease.

Skvorak KJ.

J Inherit Metab Dis. 2009 Apr;32(2):229-46. doi: 10.1007/s10545-009-1086-z. Epub 2009 Mar 9. Review.

PMID:
19263237
[PubMed - indexed for MEDLINE]
7.

Brain amino acid requirements and toxicity: the example of leucine.

Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I.

J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. Review. Erratum in: J Nutr. 2005 Aug;135(8):2009. Luhovyy, Bogdan [corrected to Luhovyy, Bohdan].

PMID:
15930465
[PubMed - indexed for MEDLINE]
9.

[Gene analysis of maple syrup urine disease (MSUD)].

Mitsubuchi H, Nobukuni Y, Hayashida Y, Ohta K, Indo Y, Akaboshi I, Endo F, Matsuda I.

Rinsho Byori. 1993 May;41(5):484-91. Review. Japanese.

PMID:
8350511
[PubMed - indexed for MEDLINE]
10.

Mechanisms responsible for regulation of branched-chain amino acid catabolism.

Harris RA, Joshi M, Jeoung NH.

Biochem Biophys Res Commun. 2004 Jan 9;313(2):391-6. Review.

PMID:
14684174
[PubMed - indexed for MEDLINE]
11.

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]
12.

Branched-chain amino acids: enzyme and substrate regulation.

Brosnan JT, Brosnan ME.

J Nutr. 2006 Jan;136(1 Suppl):207S-11S. Review.

PMID:
16365084
[PubMed - indexed for MEDLINE]
Free Article
13.

Overview of the molecular and biochemical basis of branched-chain amino acid catabolism.

Harris RA, Joshi M, Jeoung NH, Obayashi M.

J Nutr. 2005 Jun;135(6 Suppl):1527S-30S. Review.

PMID:
15930464
[PubMed - indexed for MEDLINE]
Free Article
14.

Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.

Pessoa-Pureur R, Wajner M.

J Inherit Metab Dis. 2007 Oct;30(5):664-72. Epub 2007 Jun 14. Review.

PMID:
17574538
[PubMed - indexed for MEDLINE]
15.

Structural basis of the dysfunctioning of human 2-oxo acid dehydrogenase complexes.

Hengeveld AF, de Kok A.

Curr Med Chem. 2002 Feb;9(4):499-520. Review.

PMID:
11945122
[PubMed - indexed for MEDLINE]
16.

Utility of hemodialysis in maple syrup urine disease.

Puliyanda DP, Harmon WE, Peterschmitt MJ, Irons M, Somers MJ.

Pediatr Nephrol. 2002 Apr;17(4):239-42. Review.

PMID:
11956873
[PubMed - indexed for MEDLINE]
17.

Interrupting the mechanisms of brain injury in a model of maple syrup urine disease encephalopathy.

Zinnanti WJ, Lazovic J.

J Inherit Metab Dis. 2012 Jan;35(1):71-9. doi: 10.1007/s10545-011-9333-5. Epub 2011 May 4. Review.

PMID:
21541722
[PubMed - indexed for MEDLINE]
18.

Branched-chain amino acid catabolism in exercise and liver disease.

Shimomura Y, Honda T, Shiraki M, Murakami T, Sato J, Kobayashi H, Mawatari K, Obayashi M, Harris RA.

J Nutr. 2006 Jan;136(1 Suppl):250S-3S. Review.

PMID:
16365092
[PubMed - indexed for MEDLINE]
Free Article
19.

[Maple syrup urine disease].

Indo Y, Matsuda I.

Ryoikibetsu Shokogun Shirizu. 1998;(18 Pt 1):201-4. Review. Japanese. No abstract available.

PMID:
9590028
[PubMed - indexed for MEDLINE]
20.

Maple syrup urine disease 1954 to 1993.

Peinemann F, Danner DJ.

J Inherit Metab Dis. 1994;17(1):3-15. Review. No abstract available.

PMID:
8051937
[PubMed - indexed for MEDLINE]

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