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Items: 1 to 20 of 126

1.

Phenylbutyrate improves nitrogen disposal via an alternative pathway without eliciting an increase in protein breakdown and catabolism in control and ornithine transcarbamylase-deficient patients.

Marini JC, Lanpher BC, Scaglia F, O'Brien WE, Sun Q, Garlick PJ, Jahoor F, Lee B.

Am J Clin Nutr. 2011 Jun;93(6):1248-54. doi: 10.3945/ajcn.110.009043. Epub 2011 Apr 13.

2.

Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.

Scaglia F, Carter S, O'Brien WE, Lee B.

Mol Genet Metab. 2004 Apr;81 Suppl 1:S79-85.

PMID:
15050979
3.

In vivo nitrogen metabolism in ornithine transcarbamylase deficiency.

Yudkoff M, Daikhin Y, Nissim I, Jawad A, Wilson J, Batshaw M.

J Clin Invest. 1996 Nov 1;98(9):2167-73.

4.

Acute effects of phenylbutyrate on glutamine, branched-chain amino acid and protein metabolism in skeletal muscles of rats.

Holecek M, Vodenicarovova M, Siman P.

Int J Exp Pathol. 2017 Jun;98(3):127-133. doi: 10.1111/iep.12231. Epub 2017 Jun 16.

PMID:
28621016
5.

Phenylbutyrate exerts adverse effects on liver regeneration and amino acid concentrations in partially hepatectomized rats.

Holecek M, Vodenicarovova M.

Int J Exp Pathol. 2016 Jun;97(3):278-84. doi: 10.1111/iep.12190. Epub 2016 Jul 6.

6.
7.

Restoration of nitrogen homeostasis in a man with ornithine transcarbamylase deficiency.

Brusilow SW, Finkelstien J.

Metabolism. 1993 Oct;42(10):1336-9. Erratum in: Metabolism 1994 Jul;43(7):929.

PMID:
8412748
8.

Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders.

Burrage LC, Jain M, Gandolfo L, Lee BH; Members of the Urea Cycle Disorders Consortium, Nagamani SC.

Mol Genet Metab. 2014 Sep-Oct;113(1-2):131-5. doi: 10.1016/j.ymgme.2014.06.005. Epub 2014 Jul 3.

9.

Differential utilization of systemic and enteral ammonia for urea synthesis in control subjects and ornithine transcarbamylase deficiency carriers.

Scaglia F, Marini J, Rosenberger J, Henry J, Garlick P, Lee B, Reeds P.

Am J Clin Nutr. 2003 Oct;78(4):749-55.

10.

New insights in nutritional management and amino acid supplementation in urea cycle disorders.

Scaglia F.

Mol Genet Metab. 2010;100 Suppl 1:S72-6. doi: 10.1016/j.ymgme.2010.02.019. Epub 2010 Mar 1.

11.

Acute depletion of plasma glutamine increases leucine oxidation in prednisone-treated humans.

Le Bacquer O, Mauras N, Welch S, Haymond M, Darmaun D.

Clin Nutr. 2007 Apr;26(2):231-8. Epub 2006 Nov 13.

12.

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.

13.

In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle.

Lee B, Yu H, Jahoor F, O'Brien W, Beaudet AL, Reeds P.

Proc Natl Acad Sci U S A. 2000 Jul 5;97(14):8021-6.

14.

Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency.

Nagasaka H, Yorifuji T, Murayama K, Kubota M, Kurokawa K, Murakami T, Kanazawa M, Takatani T, Ogawa A, Ogawa E, Yamamoto S, Adachi M, Kobayashi K, Takayanagi M.

Eur J Pediatr. 2006 Sep;165(9):618-24. Epub 2006 May 16.

PMID:
16703326
15.

Cross-sectional multicenter study of patients with urea cycle disorders in the United States.

Tuchman M, Lee B, Lichter-Konecki U, Summar ML, Yudkoff M, Cederbaum SD, Kerr DS, Diaz GA, Seashore MR, Lee HS, McCarter RJ, Krischer JP, Batshaw ML; Urea Cycle Disorders Consortium of the Rare Diseases Clinical Research Network.

Mol Genet Metab. 2008 Aug;94(4):397-402. doi: 10.1016/j.ymgme.2008.05.004. Epub 2008 Jun 17.

16.

Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients.

Burlina AB, Ogier H, Korall H, Trefz FK.

Mol Genet Metab. 2001 Apr;72(4):351-5.

PMID:
11286510
18.

Correction of ureagenesis after gene transfer in an animal model and after liver transplantation in humans with ornithine transcarbamylase deficiency.

Batshaw ML, Robinson MB, Ye X, Pabin C, Daikhin Y, Burton BK, Wilson JM, Yudkoff M.

Pediatr Res. 1999 Nov;46(5):588-93.

PMID:
10541323
19.

4-Phenylbutyrate modulates ubiquitination of hepatocanalicular MRP2 and reduces serum total bilirubin concentration.

Hayashi H, Mizuno T, Horikawa R, Nagasaka H, Yabuki T, Takikawa H, Sugiyama Y.

J Hepatol. 2012 May;56(5):1136-44. doi: 10.1016/j.jhep.2011.11.021. Epub 2012 Jan 13.

PMID:
22245901
20.

Inhibition of post-traumatic septic proteolysis and ureagenesis and stimulation of hepatic acute-phase protein production by branched-chain amino acid TPN.

Chiarla C, Siegel JH, Kidd S, Coleman B, Mora R, Tacchino R, Placko R, Gum M, Wiles CE 3rd, Belzberg H, et al.

J Trauma. 1988 Aug;28(8):1145-72.

PMID:
2457711

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