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Items: 1 to 20 of 345

1.

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.

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.
4.
5.

Plasma arginine and leucine kinetics and urea production rates in burn patients.

Yu YM, Young VR, Castillo L, Chapman TE, Tompkins RG, Ryan CM, Burke JF.

Metabolism. 1995 May;44(5):659-66.

PMID:
7752916
6.

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.

7.

An integrated approach to the diagnosis and prospective management of partial ornithine transcarbamylase deficiency.

Scaglia F, Zheng Q, O'Brien WE, Henry J, Rosenberger J, Reeds P, Lee B.

Pediatrics. 2002 Jan;109(1):150-2.

PMID:
11773558
8.

The nutritional management of urea cycle disorders.

Leonard JV.

J Pediatr. 2001 Jan;138(1 Suppl):S40-4;discussion S44-5.

PMID:
11148548
9.

Late-onset ornithine transcarbamylase deficiency in male patients.

Finkelstein JE, Hauser ER, Leonard CO, Brusilow SW.

J Pediatr. 1990 Dec;117(6):897-902. Erratum in: J Pediatr 1991 Feb;118(2):326.

PMID:
2246687
10.

[Influence of alimentary zinc deficiency on nitrogen elimination and enzyme activities of the urea cycle].

Roth VH.

J Anim Physiol Anim Nutr (Berl). 2001 Feb;85(1-2):45-52. German.

PMID:
11686772
11.

Contribution of dietary arginine to nitrogen utilisation and excretion in juvenile sea bass (Dicentrarchus labrax) fed diets differing in protein source.

Tulli F, Vachot C, Tibaldi E, Fournier V, Kaushik SJ.

Comp Biochem Physiol A Mol Integr Physiol. 2007 May;147(1):179-88. Epub 2007 Jan 16.

PMID:
17321177
12.

Cryptogenic hepatitis masking the diagnosis of ornithine transcarbamylase deficiency.

Zammarchi E, Donati MA, Filippi L, Resti M.

J Pediatr Gastroenterol Nutr. 1996 May;22(4):380-3.

PMID:
8732901
13.

Argininosuccinate lyase deficiency.

Nagamani SC, Erez A, Lee B.

Genet Med. 2012 May;14(5):501-7. doi: 10.1038/gim.2011.1. Epub 2012 Jan 5. Review.

14.
15.

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
16.

Conversion of [15N]ammonia into urea and amino acids in humans and the effect of nutritional status.

Weijs PJ, Calder AG, Milne E, Lobley GE.

Br J Nutr. 1996 Oct;76(4):491-9.

PMID:
8942358
17.

Liver ultrastructure in mitochondrial urea cycle enzyme deficiencies and comparison with Reye's syndrome.

Latham PS, LaBrecque DR, McReynolds JW, Klatskin G.

Hepatology. 1984 May-Jun;4(3):404-7.

PMID:
6724509
18.

Expression of ornithine-urea cycle enzymes in early life stages of air-breathing walking catfish Clarias batrachus and induction of ureogenesis under hyper-ammonia stress.

Kharbuli ZY, Datta S, Biswas K, Sarma D, Saha N.

Comp Biochem Physiol B Biochem Mol Biol. 2006 Jan;143(1):44-53. Epub 2005 Nov 3.

PMID:
16271487
19.

The phenotype of ostensibly healthy women who are carriers for ornithine transcarbamylase deficiency.

Maestri NE, Lord C, Glynn M, Bale A, Brusilow SW.

Medicine (Baltimore). 1998 Nov;77(6):389-97.

20.

Amino acid acylation: a mechanism of nitrogen excretion in inborn errors of urea synthesis.

Brusilow S, Tinker J, Batshaw ML.

Science. 1980 Feb 8;207(4431):659-61.

PMID:
6243418

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