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Results: 1 to 20 of 272

1.

Hepatocyte gene therapy in a large animal: a neonatal bovine model of citrullinemia.

Lee B, Dennis JA, Healy PJ, Mull B, Pastore L, Yu H, Aguilar-Cordova E, O'Brien W, Reeds P, Beaudet AL.

Proc Natl Acad Sci U S A. 1999 Mar 30;96(7):3981-6.

PMID:
10097149
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Evaluation of gene therapy for citrullinaemia using murine and bovine models.

Patejunas G, Lee B, Dennis JA, Healy PJ, Reeds PJ, Yu H, Frazer M, Mull B, Warman AW, Beaudet AL, O'Brien WE.

J Inherit Metab Dis. 1998;21 Suppl 1:138-50.

PMID:
9686351
[PubMed - indexed for MEDLINE]
3.

Correction of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA.

Ye X, Whiteman B, Jerebtsova M, Batshaw ML.

Gene Ther. 2000 Oct;7(20):1777-82.

PMID:
11083500
[PubMed - indexed for MEDLINE]
Free Article
4.

Generation of a mouse model for citrullinemia by targeted disruption of the argininosuccinate synthetase gene.

Patejunas G, Bradley A, Beaudet AL, O'Brien WE.

Somat Cell Mol Genet. 1994 Jan;20(1):55-60.

PMID:
8197477
[PubMed - indexed for MEDLINE]
5.

Molecular definition of bovine argininosuccinate synthetase deficiency.

Dennis JA, Healy PJ, Beaudet AL, O'Brien WE.

Proc Natl Acad Sci U S A. 1989 Oct;86(20):7947-51.

PMID:
2813370
[PubMed - indexed for MEDLINE]
Free PMC Article
6.
7.

Urea cycle disorders in Thai infants: a report of 5 cases.

Wasant P, Srisomsap C, Liammongkolkul S, Svasti J.

J Med Assoc Thai. 2002 Aug;85 Suppl 2:S720-31.

PMID:
12403252
[PubMed - indexed for MEDLINE]
8.

The human argininosuccinate synthetase locus and citrullinemia.

Beaudet AL, O'Brien WE, Bock HG, Freytag SO, Su TS.

Adv Hum Genet. 1986;15:161-96, 291-2. Review. No abstract available.

PMID:
3513483
[PubMed - indexed for MEDLINE]
9.

Correction of ornithine transcarbamylase deficiency in adult spf(ash) mice and in OTC-deficient human hepatocytes with recombinant adenoviruses bearing the CAG promoter.

Kiwaki K, Kanegae Y, Saito I, Komaki S, Nakamura K, Miyazaki JI, Endo F, Matsuda I.

Hum Gene Ther. 1996 May 1;7(7):821-30.

PMID:
8860834
[PubMed - indexed for MEDLINE]
10.

A case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase.

Nukada O, Uchiyama C, Ubuka S, Yoda T, Kobayashi K, Ichiki H, Saheki T.

Acta Paediatr Jpn. 1991 Oct;33(5):672-7.

PMID:
1799125
[PubMed - indexed for MEDLINE]
11.

Prospective management of a child with neonatal citrullinemia.

Melnyk AR, Matalon R, Henry BW, Zeller WP, Lange C.

J Pediatr. 1993 Jan;122(1):96-8.

PMID:
8419623
[PubMed - indexed for MEDLINE]
12.

Qualitative abnormality of liver argininosuccinate synthetase in a patient with citrullinemia.

Matsuda Y, Tsuji A, Katunuma N.

Adv Exp Med Biol. 1982;153:77-82. No abstract available.

PMID:
7164924
[PubMed - indexed for MEDLINE]
13.

Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis.

Brusilow SW.

J Clin Invest. 1984 Dec;74(6):2144-8.

PMID:
6511918
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Long-term correction of urea cycle disorders.

Lee B, Goss J.

J Pediatr. 2001 Jan;138(1 Suppl):S62-71. Review.

PMID:
11148551
[PubMed - indexed for MEDLINE]
15.

The sparse fur mouse as a model for gene therapy in ornithine carbamoyltransferase deficiency.

Batshaw ML, Yudkoff M, McLaughlin BA, Gorry E, Anegawa NJ, Smith IA, Hyman SL, Robinson MB.

Gene Ther. 1995 Dec;2(10):743-9.

PMID:
8750014
[PubMed - indexed for MEDLINE]
16.

Citrullinemia: early diagnosis & successful management of an otherwise lethal disorder.

Balsekar MV, Ambani LM, Bhatia RS, Shah SB, Apte BN.

Indian Pediatr. 1989 Jun;26(6):589-92. No abstract available.

PMID:
2583813
[PubMed - indexed for MEDLINE]
17.

Citrullinemia: quantitative deficiency of argininosuccinate synthetase in the liver.

Oyanagi K, Itakura Y, Tsuchiyama A, Nakao T, Nakano K, Saeki T.

Tohoku J Exp Med. 1986 Apr;148(4):385-91.

PMID:
3738904
[PubMed - indexed for MEDLINE]
Free Article
18.

Effects of glucagon on urinary excretion of urea and on plasma ammonia level in argininosuccinate synthetase deficiency.

Tamura S, Kawata S, Fukuda K, Inui Y, Kakimoto H, Ishiguro H, Namba M, Kono N, Matsuzawa Y.

J Gastroenterol. 1994 Feb;29(1):31-4.

PMID:
8199693
[PubMed - indexed for MEDLINE]
19.

Qualitative and quantitative abnormalities of argininosuccinate synthetase in citrullinemia.

Saheki T, Ueda A, Hosoya M, Kusumi K, Takada S, Tsuda M, Katsunuma T.

Clin Chim Acta. 1981 Feb 5;109(3):325-35.

PMID:
6784969
[PubMed - indexed for MEDLINE]
20.

Pancreatic secretory trypsin inhibitor gene is highly expressed in the liver of adult-onset type II citrullinemia.

Kobayashi K, Nakata M, Terazono H, Shinsato T, Saheki T.

FEBS Lett. 1995 Sep 18;372(1):69-73.

PMID:
7556646
[PubMed - indexed for MEDLINE]
Free Article

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