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Items: 1 to 20 of 114

1.

Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.

Burrage LC, Nagamani SC, Campeau PM, Lee BH.

Hum Mol Genet. 2014 Sep 15;23(R1):R1-8. doi: 10.1093/hmg/ddu123. Epub 2014 Mar 20. Review.

2.

Adipose transplant for inborn errors of branched chain amino acid metabolism in mice.

Zimmerman HA, Olson KC, Chen G, Lynch CJ.

Mol Genet Metab. 2013 Aug;109(4):345-53. doi: 10.1016/j.ymgme.2013.05.010. Epub 2013 May 30.

4.

Developmental Defects of Caenorhabditis elegans Lacking Branched-chain α-Ketoacid Dehydrogenase Are Mainly Caused by Monomethyl Branched-chain Fatty Acid Deficiency.

Jia F, Cui M, Than MT, Han M.

J Biol Chem. 2016 Feb 5;291(6):2967-73. doi: 10.1074/jbc.M115.676650. Epub 2015 Dec 18.

5.

Production and characterization of murine models of classic and intermediate maple syrup urine disease.

Homanics GE, Skvorak K, Ferguson C, Watkins S, Paul HS.

BMC Med Genet. 2006 Mar 31;7:33.

6.

Regulation of the branched-chain alpha-ketoacid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease.

Harris RA, Zhang B, Goodwin GW, Kuntz MJ, Shimomura Y, Rougraff P, Dexter P, Zhao Y, Gibson R, Crabb DW.

Adv Enzyme Regul. 1990;30:245-63.

PMID:
2403034
8.

Living related versus deceased donor liver transplantation for maple syrup urine disease.

Feier F, Schwartz IV, Benkert AR, Seda Neto J, Miura I, Chapchap P, da Fonseca EA, Vieira S, Zanotelli ML, Pinto e Vairo F, Camelo JS Jr, Margutti AV, Mazariegos GV, Puffenberger EG, Strauss KA.

Mol Genet Metab. 2016 Mar;117(3):336-43. doi: 10.1016/j.ymgme.2016.01.005. Epub 2016 Jan 12.

PMID:
26786177
9.

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. doi: 10.1093/jn/135.6.1527S. Review.

PMID:
15930464
10.

Phenylbutyrate therapy for maple syrup urine disease.

Brunetti-Pierri N, Lanpher B, Erez A, Ananieva EA, Islam M, Marini JC, Sun Q, Yu C, Hegde M, Li J, Wynn RM, Chuang DT, Hutson S, Lee B.

Hum Mol Genet. 2011 Feb 15;20(4):631-40. doi: 10.1093/hmg/ddq507. Epub 2010 Nov 23.

11.

Octanoic acid promotes branched-chain amino acid catabolisms via the inhibition of hepatic branched-chain alpha-keto acid dehydrogenase kinase in rats.

Kadota Y, Toyoda T, Hayashi-Kato M, Kitaura Y, Shimomura Y.

Metabolism. 2015 Sep;64(9):1157-64. doi: 10.1016/j.metabol.2015.05.014. Epub 2015 Jun 3.

PMID:
26104959
12.

Regulation of hepatic branched-chain α-ketoacid dehydrogenase complex in rats fed a high-fat diet.

Kadota Y, Toyoda T, Kitaura Y, Adams SH, Shimomura Y.

Obes Res Clin Pract. 2013 Dec;7(6):e439-44.

13.

Hepatocyte transplantation improves phenotype and extends survival in a murine model of intermediate maple syrup urine disease.

Skvorak KJ, Paul HS, Dorko K, Marongiu F, Ellis E, Chace D, Ferguson C, Gibson KM, Homanics GE, Strom SC.

Mol Ther. 2009 Jul;17(7):1266-73. doi: 10.1038/mt.2009.99. Epub 2009 May 12.

14.

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
15.

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.

16.

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
17.

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. doi: 10.1093/jn/136.1.243S. Review.

PMID:
16365091
18.

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
19.

Alloisoleucine differentiates the branched-chain aminoacidemia of Zucker and dietary obese rats.

Olson KC, Chen G, Xu Y, Hajnal A, Lynch CJ.

Obesity (Silver Spring). 2014 May;22(5):1212-5. doi: 10.1002/oby.20691. Epub 2014 Mar 17.

20.

Branched-Chain Amino Acids and Brain Metabolism.

Sperringer JE, Addington A, Hutson SM.

Neurochem Res. 2017 Jun;42(6):1697-1709. doi: 10.1007/s11064-017-2261-5. Epub 2017 Apr 18. Review.

PMID:
28417264

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